https://www.wikidoc.org/api.php?action=feedcontributions&user=Aida.J&feedformat=atomwikidoc - User contributions [en]2024-03-28T23:30:41ZUser contributionsMediaWiki 1.40.0https://www.wikidoc.org/index.php?title=Ventilation-perfusion_mismatch&diff=1556496Ventilation-perfusion mismatch2019-03-06T14:13:05Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{SI}}<br />
{{CMG}} '''Associate Editor(s)-in-Chief:''' {{AIDA}}<br />
<br />
== Overview ==<br />
In normal lung physiology the [[V/Q ratio]] is a measurement used to determine the efficacy and adequacy of [[ventilation]] and [[perfusion]] of the lung. [[Ventilation]] is the amount of air that reaches the lungs and [[Perfusion]] is the amount of blood flow to the lung. Any discrepancy between pulmonary blood flow and [[ventilation]] is called V/Q mismatch. Ideally [[Ventilation (physiology)|ventilation]] and [[perfusion]] should be equal with a V/Q ratio of 1, but the normal lung varies due to multiple factors such as gravity, size and patency of airways, and positioning. There is a higher perfusion at the [[Base of lung|base of the lung]] than the [[Apex of lung|apex of the lung]]. This causes a higher V/Q ratio at the apex compared to the base.<ref>{{Cite journal<br />
| author = [[Marcelo Alcantara Holanda]], [[Nathalia Parente de Sousa]], [[Luana Torres Melo]], [[Liegina Silveira Marinho]], [[Helder Veras Ribeiro-Filho]], [[Luiz Ernesto de Almeida Troncon]], [[Vasco Pinheiro Diogenes Bastos]], [[Armenio Aguiar Dos Santos]] & [[Rodrigo Jose Bezerra de Siqueira]]<br />
| title = Helping students to understand physiological aspects of regional distribution of ventilation in humans: a experience from the electrical impedance tomography<br />
| journal = [[Advances in physiology education]]<br />
| volume = 42<br />
| issue = 4<br />
| pages = 655–660<br />
| year = 2018<br />
| month = December<br />
| doi = 10.1152/advan.00086.2018<br />
| pmid = 30387699<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref>The average V/Q ratio in a normal lung is about 0.8, with about 4 liters of oxygen and 5 liters of blood entering the lung per minute.<ref>http://www.rnceus.com/abgs/abgvq.html</ref>Diseased lung can cause a V/Q mismatch due to decreased blood flow or oxygenation. This results in [[hypoxemia]], which is a decreased oxygen concentration of blood.<br />
<br />
== Causes ==<br />
Some conditions that cause decrease in V/Q are: <br />
* [[Chronic Bronchitis]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Kelvin Hsu]], [[Jonathan P. Williamson]], [[Matthew J. Peters]] & [[Alvin J. Ing]]<br />
| title = Endoscopic Lung Volume Reduction in COPD: Improvements in Gas Transfer Capacity Are Associated With Improvements in Ventilation and Perfusion Matching<br />
| journal = [[Journal of bronchology & interventional pulmonology]]<br />
| volume = 25<br />
| issue = 1<br />
| pages = 48–53<br />
| year = 2018<br />
| month = January<br />
| doi = 10.1097/LBR.0000000000000445<br />
| pmid = 29261579<br />
}}</ref><br />
* [[Asthma]]<ref>{{Cite journal<br />
| author = [[Krishnan Parameswaran]], [[Andrew C. Knight]], [[Niall P. Keaney]], [[E. David Williams]] & [[Ian K. Taylor]]<br />
| title = Ventilation and perfusion lung scintigraphy of allergen-induced airway responses in atopic asthmatic subjects<br />
| journal = [[Canadian respiratory journal]]<br />
| volume = 14<br />
| issue = 5<br />
| pages = 285–291<br />
| year = 2007<br />
| month = July-August<br />
| doi = 10.1155/2007/474202<br />
| pmid = 17703244<br />
}}</ref><br />
* [[Foreign body aspiration]]<ref>{{Cite journal<br />
| author = [[Natan Cramer]], [[Roger S.. Taylor]] & [[Melissa M.. Tavarez]]<br />
| title = Foreign Body Aspiration<br />
| year = 2018<br />
| month = January<br />
| pmid = 30285375<br />
}}</ref><br />
* [[Hepatopulmonary syndrome]]<br />
* [[ARDS]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* Bronchiectasis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Interstitial Lung disease<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Cystic Fibrosis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Pulmonary Hypertension<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
Some conditions that cause increase in V/Q are:<br />
* [[Pulmonary embolism]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* [[Emphysema]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
<br />
<br />
Extreme conditions:<br />
* An area of no ventilation is termed a shunt<br />
**V/Q ratio= 0<br />
* An area of no perfusion is termed a dead space<br />
**V/Q ratio is undefined<br />
<br />
==Pathogenesis==<br />
<br />
V/Q mismatch is one of the most common causes of hypoxemia. It can be caused by [[Obstructive lung disease|obstructive lung]] diseases, pulmonary vascular diseases, and [[Interstitial lung disease|interstitial diseases]]. Anything that affects the blood flow and oxygenation in the lung can cause a V/Q mismatch. An increased V/Q mismatch is caused by a decrease in blood flow to the lung, for example a [[pulmonary embolism]]. A decreased V/Q mismatch is caused by a decrease in ventilation or an airway obstruction, for example [[Asthma]]. A V/Q mismatch due to a perfusion defect will improve with 100% [[Oxygen therapy|oxygen therapy.]]<ref>{{Cite journal<br />
| author = [[Vu M. Mai]], [[Benjamin Liu]], [[Jason A. Polzin]], [[Wei Li]], [[Saban Kurucay]], [[Alexander A. Bankier]], [[Jack Knight-Scott]], [[Priti Madhav]], [[Robert R. Edelman]] & [[Qun Chen]]<br />
| title = Ventilation-perfusion ratio of signal intensity in human lung using oxygen-enhanced and arterial spin labeling techniques<br />
| journal = [[Magnetic resonance in medicine]]<br />
| volume = 48<br />
| issue = 2<br />
| pages = 341–350<br />
| year = 2002<br />
| month = August<br />
| doi = 10.1002/mrm.10230<br />
| pmid = 12210943<br />
}}</ref> <ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref> In normal conditions when there is a low ventilation, the body tries to keep this ratio in a normal range by restricting the perfusion in that specific area of the lung. This unique mechanism is called hypoxic pulmonary vasoconstriction. If this process continues for a long time it can cause pulmonary hypertension.<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
<br />
==Associated Conditions==<br />
Some conditions that cause decrease in V/Q are: <br />
* [[Chronic Bronchitis]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Kelvin Hsu]], [[Jonathan P. Williamson]], [[Matthew J. Peters]] & [[Alvin J. Ing]]<br />
| title = Endoscopic Lung Volume Reduction in COPD: Improvements in Gas Transfer Capacity Are Associated With Improvements in Ventilation and Perfusion Matching<br />
| journal = [[Journal of bronchology & interventional pulmonology]]<br />
| volume = 25<br />
| issue = 1<br />
| pages = 48–53<br />
| year = 2018<br />
| month = January<br />
| doi = 10.1097/LBR.0000000000000445<br />
| pmid = 29261579<br />
}}</ref><br />
* [[Asthma]]<ref>{{Cite journal<br />
| author = [[Krishnan Parameswaran]], [[Andrew C. Knight]], [[Niall P. Keaney]], [[E. David Williams]] & [[Ian K. Taylor]]<br />
| title = Ventilation and perfusion lung scintigraphy of allergen-induced airway responses in atopic asthmatic subjects<br />
| journal = [[Canadian respiratory journal]]<br />
| volume = 14<br />
| issue = 5<br />
| pages = 285–291<br />
| year = 2007<br />
| month = July-August<br />
| doi = 10.1155/2007/474202<br />
| pmid = 17703244<br />
}}</ref><br />
* [[Foreign body aspiration]]<ref>{{Cite journal<br />
| author = [[Natan Cramer]], [[Roger S.. Taylor]] & [[Melissa M.. Tavarez]]<br />
| title = Foreign Body Aspiration<br />
| year = 2018<br />
| month = January<br />
| pmid = 30285375<br />
}}</ref><br />
* [[Hepatopulmonary syndrome]]<ref>{{Cite journal<br />
| author = [[Zulkifli Amin]], [[Hilman Zulkifli Amin]] & [[Nadim Marchian Tedyanto]]<br />
| title = Hepatopulmonary Syndrome: A Brief Review<br />
| journal = [[Romanian journal of internal medicine = Revue roumaine de medecine interne]]<br />
| volume = 54<br />
| issue = 2<br />
| pages = 93–97<br />
| year = 2016<br />
| month = April-June<br />
| doi = 10.1515/rjim-2016-0015<br />
| pmid = 27352437<br />
}}</ref><br />
* [[ARDS]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* Bronchiectasis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Interstitial Lung disease<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Cystic Fibrosis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Pulmonary Hypertension<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
Some conditions that cause increase in V/Q are:<br />
* [[Pulmonary embolism]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* [[Emphysema]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
<br />
<br />
Extreme conditions:<br />
* An area of no ventilation is termed a shunt<br />
**V/Q ratio= 0<br />
* An area of no perfusion is termed a dead space<br />
**V/Q ratio is undefined<br />
<br />
==Genetics==<br />
The association between V/Q mismatch and genetics depends on the etiology of the mismatch. Some diseases with genetic components include:<br />
*[[Asthma]] has over 100 genetic associations.<ref>{{Cite journal<br />
| author = [[C. Ober]] & [[S. Hoffjan]]<br />
| title = Asthma genetics 2006: the long and winding road to gene discovery<br />
| journal = [[Genes and immunity]]<br />
| volume = 7<br />
| issue = 2<br />
| pages = 95–100<br />
| year = 2006<br />
| month = March<br />
| doi = 10.1038/sj.gene.6364284<br />
| pmid = 16395390<br />
}}</ref><br />
*[[Emphysema]] can be associated with a deficiency of alpha 1 antitrypsin <ref>{{Cite journal<br />
| author = [[James J. Tasch]], [[Ann T. McLaughlan]] & [[Asad A. Nasir]]<br />
| title = A Novel Approach to Screening for Alpha-1 Antitrypsin Deficiency: Inpatient Testing at a Teaching Institution<br />
| journal = [[Chronic obstructive pulmonary diseases (Miami, Fla.)]]<br />
| volume = 5<br />
| issue = 2<br />
| pages = 106–110<br />
| year = 2018<br />
| month = April<br />
| doi = 10.15326/jcopdf.5.2.2017.0170<br />
| pmid = 30374448<br />
}}</ref><br />
*[[Cystic fibrosis]] is a genetic disorder affecting chloride transporters <ref>{{Cite journal<br />
| author = [[Shuzhong Zhang]], [[Chandra L. Shrestha]] & [[Benjamin T. Kopp]]<br />
| title = Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function<br />
| journal = [[Scientific reports]]<br />
| volume = 8<br />
| issue = 1<br />
| pages = 17066<br />
| year = 2018<br />
| month = November<br />
| doi = 10.1038/s41598-018-35151-7<br />
| pmid = 30459435<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Zhe Zhang]], [[Fangyu Liu]] & [[Jue Chen]]<br />
| title = Molecular structure of the ATP-bound, phosphorylated human CFTR<br />
| journal = [[Proceedings of the National Academy of Sciences of the United States of America]]<br />
| year = 2018<br />
| month = November<br />
| doi = 10.1073/pnas.1815287115<br />
| pmid = 30459277<br />
}}</ref><br />
<br />
== Gross Pathology ==<br />
The gross pathology depends on the exact reason for the V/Q mismatch. For example:<br />
*Pulmonary fibrosis can present with a honeycomb appearance of the lung <ref>{{Cite journal<br />
| author = [[Hiroaki Arakawa]] & [[Koichi Honma]]<br />
| title = Honeycomb lung: history and current concepts<br />
| journal = [[AJR. American journal of roentgenology]]<br />
| volume = 196<br />
| issue = 4<br />
| pages = 773–782<br />
| year = 2011<br />
| month = April<br />
| doi = 10.2214/AJR.10.4873<br />
| pmid = 21427324<br />
}}</ref><br />
* Pulmonary infarct can present as a wedge shaped infarct <ref>{{Cite journal<br />
| author = [[Massimo Miniati]], [[Matteo Bottai]], [[Cesario Ciccotosto]], [[Luca Roberto]] & [[Simonetta Monti]]<br />
| title = Predictors of Pulmonary Infarction<br />
| journal = [[Medicine]]<br />
| volume = 94<br />
| issue = 41<br />
| pages = e1488<br />
| year = 2015<br />
| month = October<br />
| doi = 10.1097/MD.0000000000001488<br />
| pmid = 26469892<br />
}}</ref><br />
<br />
== Microscopic Pathology ==<br />
The microscopic pathology depends on the exact reason for the V/Q mismatch. For example:<br />
*In [[asthma]] there are extracellular Charcot-Leyden crystals and increased mucosal [[Goblet cell|goblet]] cells<ref>{{Cite journal<br />
| author = [[P. J. Dor]], [[S. J. Ackerman]] & [[G. J. Gleich]]<br />
| title = Charcot-Leyden crystal protein and eosinophil granule major basic protein in sputum of patients with respiratory diseases<br />
| journal = [[The American review of respiratory disease]]<br />
| volume = 130<br />
| issue = 6<br />
| pages = 1072–1077<br />
| year = 1984<br />
| month = December<br />
| doi = 10.1164/arrd.1984.130.6.1072<br />
| pmid = 6508005<br />
}}</ref><br />
*In Interstitial lung disease caused by asbestosis ferruginous bodies may be present <ref>{{Cite journal<br />
| author = [[A. M. Churg]] & [[M. L. Warnock]]<br />
| title = Asbestos and other ferruginous bodies: their formation and clinical significance<br />
| journal = [[The American journal of pathology]]<br />
| volume = 102<br />
| issue = 3<br />
| pages = 447–456<br />
| year = 1981<br />
| month = March<br />
| pmid = 6101235<br />
}}</ref><br />
<br />
== Understanding V/Q mismatch in the context of hypoxia ==<br />
There are two causes of V/Q mismatch:<br />
# Decreased Ventilation<br />
# Decreased Perfusion<br />
=== Shunts ===<br />
Pulmonary shunts occur when the V/Q ratio is zero. It is formed when there is absent ventilation in one part of the lung with normal perfusion. <ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref> This deoxygenated blood enters arterial circulation without getting oxygenated in the lung. Absorptive or compressive pulmonary atelectasis is the major reason for shunt formation. Pulmonary AV malformation, hepatopulmonary syndrome <ref>{{Cite journal<br />
| author = [[Zulkifli Amin]], [[Hilman Zulkifli Amin]] & [[Nadim Marchian Tedyanto]]<br />
| title = Hepatopulmonary Syndrome: A Brief Review<br />
| journal = [[Romanian journal of internal medicine = Revue roumaine de medecine interne]]<br />
| volume = 54<br />
| issue = 2<br />
| pages = 93–97<br />
| year = 2016<br />
| month = April-June<br />
| doi = 10.1515/rjim-2016-0015<br />
| pmid = 27352437<br />
}}</ref>, ARDS, and pulmonary edema are less common causes. Shunting is characterized by it poor response to 100% oxygen therapy.<br />
<br />
=== Dead space ventilation ===<br />
Dead space ventilation occurs when V/Q ratio is undefined. When blood supply to part of the lung is cut off, oxygen in the ventilated atmospheric air is not able to enter the bloodstream leading to lesser overall efficiency of the alveolar oxygenation mechanism. <ref>{{Cite journal<br />
| author = [[Sal Intagliata]] & [[Alessandra Rizzo]]<br />
| title = Physiology, Lung Dead Space<br />
| year = 2018<br />
| month = January<br />
| pmid = 29494107<br />
}}</ref> Pulmonary Embolism is the most common cause of dead space ventilation.<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref> Hypoxemia caused by dead space ventilation will improve with 100% oxygen therapy.<br />
<br />
== Natural History and Complications ==<br />
<br />
If left untreated patients with V/Q mismatch may progress to develop [[pulmonary hypertension]] <ref>{{Cite journal<br />
| author = [[Michael Y. Shino]], [[Joseph P. 3rd Lynch]], [[Rajeev Saggar]], [[Fereidoun Abtin]], [[John A. Belperio]] & [[Rajan Saggar]]<br />
| title = Pulmonary hypertension complicating interstitial lung disease and COPD<br />
| journal = [[Seminars in respiratory and critical care medicine]]<br />
| volume = 34<br />
| issue = 5<br />
| pages = 600–619<br />
| year = 2013<br />
| month = October<br />
| doi = 10.1055/s-0033-1356548<br />
| pmid = 24037628<br />
}}</ref>, [[respiratory failure]], and even [[death]]. <ref>{{Cite journal<br />
| author = [[Kelly Stam]], [[Richard W. B. van Duin]], [[Andre Uitterdijk]], [[Ilona Krabbendam-Peters]], [[Oana Sorop]], [[A. H. Jan Danser]], [[Dirk J. Duncker]] & [[Daphne Merkus]]<br />
| title = Pulmonary microvascular remodeling in chronic thrombo-embolic pulmonary hypertension<br />
| journal = [[American journal of physiology. Lung cellular and molecular physiology]]<br />
| year = 2018<br />
| month = September<br />
| doi = 10.1152/ajplung.00043.2018<br />
| pmid = 30260284<br />
}}</ref> <ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref> In patients with an decreased V/Q ratio, 100% oxygen can be used as acute treatment while additional diagnostic studies are necessary to determine the underlying cause.<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref> Prognosis, mortality and survival rate of patients with V/Q mismatch depend on the underlying cause and early treatment.<ref>{{Cite journal<br />
| author = [[Brian N. Jobse]], [[Cory Ajr McCurry]], [[Mathieu C. Morissette]], [[Rod G. Rhem]], [[Martin R. Stampfli]] & [[Nancy Renee Labiris]]<br />
| title = Impact of inflammation, emphysema, and smoking cessation on V/Q in mouse models of lung obstruction<br />
| journal = [[Respiratory research]]<br />
| volume = 15<br />
| pages = 42<br />
| year = 2014<br />
| month = April<br />
| doi = 10.1186/1465-9921-15-42<br />
| pmid = 24730756<br />
}}</ref><br />
<br />
== History, Symptoms, and Physical Exam ==<br />
Symptoms of V/Q mismatch depend on the underlying cause but in general a majority of cases with V/Q mismatch include:<br />
*[[Dyspnea]]<br />
*[[Exercise intolerance]]<br />
*[[Chest pain]]<br />
*[[Dizziness]]<br />
*[[Altered mental status]]<br />
*[[Cough]]<br />
<br />
Patients with V/Q mismatch usually appear distressed. Physical examination of patients with V/Q mismatch depends on the underlying cause. <br />
Some common physical examination findings of V/Q mismatch include:<br />
*[[Tachypnea]] or [[Bradypnea]]<br />
*Abnormal or decreased [[Breath sounds]]<br />
*Rapid pulse.<br />
If the patient is in [[respiratory failure]] they may present with: <br />
*Visible internal jugular venous pulse<br />
*[[Intercostal retractions]]<br />
*[[Pallor]]<br />
*[[Cyanosis]]<br />
*Nasal flaring<br />
<br />
== Differential Diagnosis ==<br />
V/Q mismatch is finding that can be indicative of a serious respiratory disease. The differential diagnosis for V/Q mismatch includes:<br />
<br />
* [[Chronic Bronchitis]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><ref>{{Cite journal<br />
| author = [[Kelvin Hsu]], [[Jonathan P. Williamson]], [[Matthew J. Peters]] & [[Alvin J. Ing]]<br />
| title = Endoscopic Lung Volume Reduction in COPD: Improvements in Gas Transfer Capacity Are Associated With Improvements in Ventilation and Perfusion Matching<br />
| journal = [[Journal of bronchology & interventional pulmonology]]<br />
| volume = 25<br />
| issue = 1<br />
| pages = 48–53<br />
| year = 2018<br />
| month = January<br />
| doi = 10.1097/LBR.0000000000000445<br />
| pmid = 29261579<br />
}}</ref><br />
* [[Asthma]]<ref>{{Cite journal<br />
| author = [[Krishnan Parameswaran]], [[Andrew C. Knight]], [[Niall P. Keaney]], [[E. David Williams]] & [[Ian K. Taylor]]<br />
| title = Ventilation and perfusion lung scintigraphy of allergen-induced airway responses in atopic asthmatic subjects<br />
| journal = [[Canadian respiratory journal]]<br />
| volume = 14<br />
| issue = 5<br />
| pages = 285–291<br />
| year = 2007<br />
| month = July-August<br />
| doi = 10.1155/2007/474202<br />
| pmid = 17703244<br />
}}</ref><br />
* [[Foreign body aspiration]]<ref>{{Cite journal<br />
| author = [[Natan Cramer]], [[Roger S.. Taylor]] & [[Melissa M.. Tavarez]]<br />
| title = Foreign Body Aspiration<br />
| year = 2018<br />
| month = January<br />
| pmid = 30285375<br />
}}</ref><br />
* [[Hepatopulmonary syndrome]]<ref>{{Cite journal<br />
| author = [[Zulkifli Amin]], [[Hilman Zulkifli Amin]] & [[Nadim Marchian Tedyanto]]<br />
| title = Hepatopulmonary Syndrome: A Brief Review<br />
| journal = [[Romanian journal of internal medicine = Revue roumaine de medecine interne]]<br />
| volume = 54<br />
| issue = 2<br />
| pages = 93–97<br />
| year = 2016<br />
| month = April-June<br />
| doi = 10.1515/rjim-2016-0015<br />
| pmid = 27352437<br />
}}</ref><br />
* [[ARDS]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* Bronchiectasis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Interstitial Lung disease<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Cystic Fibrosis<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* Pulmonary Hypertension<ref>{{Cite journal<br />
| author = [[Malay Sarkar]], [[N. Niranjan]] & [[P. K. Banyal]]<br />
| title = Mechanisms of hypoxemia<br />
| journal = [[Lung India : official organ of Indian Chest Society]]<br />
| volume = 34<br />
| issue = 1<br />
| pages = 47–60<br />
| year = 2017<br />
| month = January-February<br />
| doi = 10.4103/0970-2113.197116<br />
| pmid = 28144061<br />
}}</ref><br />
* [[Pulmonary embolism]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
* [[Emphysema]]<ref>{{Cite journal<br />
| author = [[Johan Petersson]] & [[Robb W. Glenny]]<br />
| title = Gas exchange and ventilation-perfusion relationships in the lung<br />
| journal = [[The European respiratory journal]]<br />
| volume = 44<br />
| issue = 4<br />
| pages = 1023–1041<br />
| year = 2014<br />
| month = October<br />
| doi = 10.1183/09031936.00037014<br />
| pmid = 25063240<br />
}}</ref><br />
A workup must be done to diagnose and treat the underlying illness<br />
<br />
== Work up ==<br />
V/Q mismatch can be caused by various diseases and a workup must be done for diagnosis and treatment.<br />
*Labs:<br />
** [[Arterial Blood Gas]]<br />
**[[PAO2]]<br />
**[[PaO2]]<br />
**[[PaCo2]]<br />
**[[Bicarbonate levels]]<br />
**[[DLCO2]]<br />
**[[Spirometry]]<br />
<br />
*Imaging<br />
**[[Chest X-Ray]]<br />
**[[Ventilation-Perfusion scan]]<br />
<br />
<br />
Calculations using measurements from Arterial Blood Gas (ABG) and the response of those measures to supplemental oxygen are used to investigate the cause of hypoxia. <br />
{| class="wikitable"<br />
|+Physiological changes in Alveolar and Blood gas in various causes of hypoxia<br />
!Cause<br />
!P(Alv)O2<br />
!A-a gradient<br />
!Response to <br />
supplemental oxygen<br />
|-<br />
|Diffusion limitation<br />
|Normal<br />
|Increased<br />
|Improved PaO2<br />
|-<br />
|Hypoventilation<br />
|Reduced<br />
|Normal<br />
|Improved PaO2<br />
|-<br />
|Reduced PiO2<br />
|Reduced<br />
|Normal<br />
|Improved PaO2<br />
|-<br />
|Shunt formation<br />
|Reduced in local areas of lung<br />
|Increased<br />
|Improved PaO2<br />
|-<br />
|Dead space formation<br />
|Normal<br />
|Increased<br />
|Minimal to no improvement<br />
|}<br />
PiO2 - partial pressure of oxygen in inspired air<br />
<br />
P(Alv)O2 - partial pressure of oxygen in alveolar air<br />
<br />
PaO2 - partial pressure of oxygen in arterial air<br />
<br />
A-a gradient - P(Alv)O2 - PaO2<br />
<br />
== References ==<br />
{{Reflist|2}}<br />
<references /></div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537828Urethral cancer pathophysiology2019-01-21T01:18:17Z<p>Aida.J: /* Genetics */</p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref><ref name="pmid28776126">{{cite journal |vauthors=Browne BM, Vanni AJ |title=Management of Urethral Stricture and Bladder Neck Contracture Following Primary and Salvage Treatment of Prostate Cancer |journal=Curr Urol Rep |volume=18 |issue=10 |pages=76 |date=October 2017 |pmid=28776126 |doi=10.1007/s11934-017-0729-0 |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
Also, overexpression of the semaphorin 3A in patients with [[urethral]] cancer has been reported <ref name="pmid29288007">{{cite journal |vauthors=Vadasz Z, Rubinstein J, Bejar J, Sheffer H, Halachmi S |title=Overexpression of semaphorin 3A in patients with urothelial cancer |journal=Urol. Oncol. |volume=36 |issue=4 |pages=161.e1–161.e6 |date=April 2018 |pmid=29288007 |doi=10.1016/j.urolonc.2017.12.007 |url=}}</ref>.<br />
<br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type, they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]] <ref name="pmid29516944">{{cite journal |vauthors=Mallya V, Mallya A, Gayathri J |title=Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature |journal=J Cancer Res Ther |volume=14 |issue=2 |pages=468–470 |date=2018 |pmid=29516944 |doi=10.4103/0973-1482.226734 |url=}}</ref>: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537827Urethral cancer pathophysiology2019-01-21T01:17:12Z<p>Aida.J: /* Genetics */</p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref><ref name="pmid28776126">{{cite journal |vauthors=Browne BM, Vanni AJ |title=Management of Urethral Stricture and Bladder Neck Contracture Following Primary and Salvage Treatment of Prostate Cancer |journal=Curr Urol Rep |volume=18 |issue=10 |pages=76 |date=October 2017 |pmid=28776126 |doi=10.1007/s11934-017-0729-0 |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
Also, overexpression of the semaphorin 3A in patients with [[urethral]] cancer has been reported.<br />
<br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type, they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]] <ref name="pmid29516944">{{cite journal |vauthors=Mallya V, Mallya A, Gayathri J |title=Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature |journal=J Cancer Res Ther |volume=14 |issue=2 |pages=468–470 |date=2018 |pmid=29516944 |doi=10.4103/0973-1482.226734 |url=}}</ref>: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537826Urethral cancer pathophysiology2019-01-21T01:11:53Z<p>Aida.J: /* Gross Pathology */</p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref><ref name="pmid28776126">{{cite journal |vauthors=Browne BM, Vanni AJ |title=Management of Urethral Stricture and Bladder Neck Contracture Following Primary and Salvage Treatment of Prostate Cancer |journal=Curr Urol Rep |volume=18 |issue=10 |pages=76 |date=October 2017 |pmid=28776126 |doi=10.1007/s11934-017-0729-0 |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type, they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]] <ref name="pmid29516944">{{cite journal |vauthors=Mallya V, Mallya A, Gayathri J |title=Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature |journal=J Cancer Res Ther |volume=14 |issue=2 |pages=468–470 |date=2018 |pmid=29516944 |doi=10.4103/0973-1482.226734 |url=}}</ref>: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537825Urethral cancer pathophysiology2019-01-21T01:10:48Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref><ref name="pmid28776126">{{cite journal |vauthors=Browne BM, Vanni AJ |title=Management of Urethral Stricture and Bladder Neck Contracture Following Primary and Salvage Treatment of Prostate Cancer |journal=Curr Urol Rep |volume=18 |issue=10 |pages=76 |date=October 2017 |pmid=28776126 |doi=10.1007/s11934-017-0729-0 |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]] <ref name="pmid29516944">{{cite journal |vauthors=Mallya V, Mallya A, Gayathri J |title=Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature |journal=J Cancer Res Ther |volume=14 |issue=2 |pages=468–470 |date=2018 |pmid=29516944 |doi=10.4103/0973-1482.226734 |url=}}</ref>: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537824Urethral cancer pathophysiology2019-01-21T01:08:00Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]] <ref name="pmid29516944">{{cite journal |vauthors=Mallya V, Mallya A, Gayathri J |title=Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature |journal=J Cancer Res Ther |volume=14 |issue=2 |pages=468–470 |date=2018 |pmid=29516944 |doi=10.4103/0973-1482.226734 |url=}}</ref>: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1537823Urethral cancer pathophysiology2019-01-21T01:04:33Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men.<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1536030Urethral cancer pathophysiology2019-01-15T17:20:11Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are <ref name="pmid25347253">{{cite journal |vauthors=Klemann N, Toft BG, Thind P |title=[Urethral cancer is rare and difficult to diagnose] |language=Danish |journal=Ugeskr. Laeg. |volume=176 |issue=4A |pages=V07130435 |date=January 2014 |pmid=25347253 |doi= |url=}}</ref>:<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men/<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1536027Urethral cancer pathophysiology2019-01-15T17:09:06Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref> <ref name="pmid27717437">{{cite journal |vauthors=Zargar-Shoshtari K, Sexton WJ, Poch MA |title=Management of Urethral Recurrences: Urothelial and Nonurothelial |journal=Urol. Clin. North Am. |volume=43 |issue=4 |pages=515–521 |date=November 2016 |pmid=27717437 |doi=10.1016/j.ucl.2016.06.012 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men/<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1536025Urethral cancer pathophysiology2019-01-15T17:07:48Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type <ref name="pmid27720774">{{cite journal |vauthors=Sui W, RoyChoudhury A, Wenske S, Decastro GJ, McKiernan JM, Anderson CB |title=Outcomes and Prognostic Factors of Primary Urethral Cancer |journal=Urology |volume=100 |issue= |pages=180–186 |date=February 2017 |pmid=27720774 |doi=10.1016/j.urology.2016.09.042 |url=}}</ref>.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men/<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1536015Urethral cancer pathophysiology2019-01-15T16:52:39Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men/<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Adenocarcinoma in the [[urethra]] is commonly associated with [[Diverticular disease|diverticula]] and [[prostatic]] adenocarcinoma.<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20. <br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535168Urethral cancer pathophysiology2019-01-12T19:44:26Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Associated Conditions ==<br />
* Urethral cancers in proximal urethra have worse prognosis than those arising in the distal portion in men/<br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20.<br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535167Urethral cancer pathophysiology2019-01-12T19:38:51Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Although cigarette smoking can cause bladder cancer but the role of it in causing primary urethral cancer is still unknown.<br />
<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20.<br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535166Urethral cancer pathophysiology2019-01-12T19:28:11Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges. Stains: High molecular weight cytokeratin (CK903, CK5/6), p63, p16.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools. Stains: P53, CK20.<br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535165Urethral cancer pathophysiology2019-01-12T19:26:43Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::**[[SCC]]: high [[mitotic]] activity, nuclear atypia. Positive with cytoplasmic beta-catenin stain. pleomorphic tumor cells with focal or abundant [[keratinization]], intercellular bridges.<br />
::**[[Adenocarcinoma]]: simple or [[Pseudostratified columnar|pseudostratified]] columnar epithelium with [[hyperchromatic]] nuclei. vacuolated cytoplasm with mucin pools.<br />
::**[[Clear Cell]]: clear or [[eosinophilic]] cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with [[p53]] and vimentin stain. Hobnail changes and extracellular mucoid material.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535164Urethral cancer pathophysiology2019-01-12T17:39:20Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
Also the pathogenesis is different in male and female. <br />
<br />
In male [[Prostate|prostatic]] and membranous portions of the urethra cancer are more from [[bladder cancer]]. Bulbous and membranous portions urethral cancers are most commonly [[squamous cell carcinoma]].<br />
<br />
In female proximal 2/3 of the urethral cancer are more primary types and distal 1/3 is usually squamous cell carcinoma.<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::* [[SCC]]: [[keratinization]], nuclear atypia. Positive with cytoplasmic beta-catenin stain.<br />
::* Adenocarcinoma: Columnar epithelium with [[hyperchromatic]] nuclei.<br />
::* [[Clear Cell]]: clear or eosinophilic cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with p53 and vimentin stain.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535163Urethral cancer pathophysiology2019-01-12T17:30:10Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer. Other etiologies for primary types are:<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::* [[SCC]]: [[keratinization]], nuclear atypia. Positive with cytoplasmic beta-catenin stain.<br />
::* Adenocarcinoma: Columnar epithelium with [[hyperchromatic]] nuclei.<br />
::* [[Clear Cell]]: clear or eosinophilic cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with p53 and vimentin stain.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535162Urethral cancer pathophysiology2019-01-12T17:29:07Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. In primary type of the urethral cancer It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer.<br />
<br />
<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::* [[SCC]]: [[keratinization]], nuclear atypia. Positive with cytoplasmic beta-catenin stain.<br />
::* Adenocarcinoma: Columnar epithelium with [[hyperchromatic]] nuclei.<br />
::* [[Clear Cell]]: clear or eosinophilic cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with p53 and vimentin stain.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535161Urethral cancer pathophysiology2019-01-12T17:28:04Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from [[bladder cancer]] which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer.<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::* [[SCC]]: [[keratinization]], nuclear atypia. Positive with cytoplasmic beta-catenin stain.<br />
::* Adenocarcinoma: Columnar epithelium with [[hyperchromatic]] nuclei.<br />
::* [[Clear Cell]]: clear or eosinophilic cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with p53 and vimentin stain.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Urethral_cancer_pathophysiology&diff=1535160Urethral cancer pathophysiology2019-01-12T17:27:18Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Urethral cancer}}<br />
{{CMG}}; {{AE}}{{Vbe}}, {{AIDA}} <br />
<br />
==Overview==<br />
Urethral cancer is a rare disease. The [[pathophysiology]] of [[Urethral cancer|urethral]] [[cancer]] depends on the [[histological]] subtypes. It could be primary from epithelial origin or secondary like from bladder cancer which is more common than primary type.<br />
<br />
== Pathogenesis ==<br />
[[Mucous|Mucous cell]]<nowiki/>s in the [[urethra]] have the ability to turnover rapidly. It has been suggested that defect in [[DNA repair]] mechanism may cause urethral cancer.<br />
<br />
Chronic inflammation and strictures: May happen after any surgery on urethra like [[urethroplasty]] <ref name="pmid916053">{{cite journal |vauthors=Colapinto V, Evans DH |title=Primary carcinoma of the male urethra developing after urethroplasty for stricture |journal=J. Urol. |volume=118 |issue=4 |pages=581–4 |date=October 1977 |pmid=916053 |doi= |url=}}</ref>. <br />
* Infection: [[Sexually transmitted disease|sexually transmitted diseases]] like [[HPV]] type 16 <ref name="pmid8886059">{{cite journal |vauthors=Cupp MR, Malek RS, Goellner JR, Espy MJ, Smith TF |title=Detection of human papillomavirus DNA in primary squamous cell carcinoma of the male urethra |journal=Urology |volume=48 |issue=4 |pages=551–5 |date=October 1996 |pmid=8886059 |doi=10.1016/S0090-4295(96)00246-4 |url=}}</ref>. <br />
<br />
* External [[radiation therapy]] <ref name="pmid12588611">{{cite journal |vauthors=Mohan H, Bal A, Punia RP, Bawa AS |title=Squamous cell carcinoma of the prostate |journal=Int. J. Urol. |volume=10 |issue=2 |pages=114–6 |date=February 2003 |pmid=12588611 |doi= |url=}}</ref><br />
<br />
* [[Urethral diverticulum|Urethral diverticula]] in female <ref name="pmid19649767">{{cite journal |vauthors=Ahmed K, Dasgupta R, Vats A, Nagpal K, Ashrafian H, Kaj B, Athanasiou T, Dasgupta P, Khan MS |title=Urethral diverticular carcinoma: an overview of current trends in diagnosis and management |journal=Int Urol Nephrol |volume=42 |issue=2 |pages=331–41 |date=June 2010 |pmid=19649767 |doi=10.1007/s11255-009-9618-x |url=}}</ref><br />
<br />
* Other: [[Arsenic]] ingestion<br />
<br />
== Genetics ==<br />
The exact gene and mutations that cause urethral cancer are unlnown. <br />
<br />
[[Mutation]] in TERT promoter, [[PAX8]], [[GATA3]], P63, P40, [[p53]] may play role in sarcomatoid urothelial carcinoma <ref name="pmid28052688">{{cite journal |vauthors=Wang X, Lopez-Beltran A, Osunkoya AO, Wang M, Zhang S, Davidson DD, Emerson RE, Williamson SR, Tan PH, Kaimakliotis HZ, Baldridge LA, MacLennan GT, Montironi R, Cheng L |title=TERT promoter mutation status in sarcomatoid urothelial carcinomas of the upper urinary tract |journal=Future Oncol |volume=13 |issue=8 |pages=705–714 |date=April 2017 |pmid=28052688 |doi=10.2217/fon-2016-0414 |url=}}</ref>. <br />
<br />
[[Fragile histidine triad]] ([[FHIT]]) gene may play a role in causing [[Bladder|bladde]]<nowiki/>r urothelial carcinoma <ref name="pmid22613411">{{cite journal |vauthors=Zhang CT, Lu R, Lin YL, Liu RL, Zhang ZH, Yang K, Dang RF, Zhang HT, Shen YG, Kong PZ, Ren HL, Li XL, Quan W, Xu Y |title=The significance of fragile histidine triad protein as a molecular prognostic marker of bladder urothelial carcinoma |journal=J. Int. Med. Res. |volume=40 |issue=2 |pages=507–16 |date=2012 |pmid=22613411 |doi=10.1177/147323001204000212 |url=}}</ref>. <br />
<br />
== Gross Pathology ==<br />
In end stage type they may appear as an exophytic mass.<br />
<br />
==Microscopic Pathology==<br />
The microscopic view of [[urethral cancer]] is depended on the location of teh cancer. The type of the cancer in the distal part of the urethra is usually [[squamous cell]]. <ref name="pmid23116581">{{cite journal |vauthors=Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ |title=Urethral cancer |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1291–314 |date=December 2012 |pmid=23116581 |doi=10.1016/j.hoc.2012.08.006 |url=}}</ref><br />
* [[Female]]<ref name="pmid23608423">{{cite journal |vauthors=Cantiello F, Cicione A, Salonia A, Autorino R, Tucci L, Madeo I, Damiano R |title=Periurethral fibrosis secondary to prostatic inflammation causing lower urinary tract symptoms: a prospective cohort study |journal=Urology |volume=81 |issue=5 |pages=1018–23 |date=May 2013 |pmid=23608423 |doi=10.1016/j.urology.2013.01.053 |url=}}</ref><br />
:* The [[female]] [[urethra]] is lined by [[transitional cell]] [[mucosa]] proximally and [[stratified]] [[squamous]] cells distally. <br />
:* Therefore, [[transitional cell carcinoma]] is most common in the [[proximal]] [[urethra]] <br />
:* [[Adenocarcinoma]] may occur in both locations and arises from [[metaplasia]] of the numerous [[Periurethral phlegmon|periurethral]] [[glands]].<br />
<br />
* [[Male]]<br />
:* The [[male]] [[urethra]] is lined by [[transitional]] cells in its [[prostatic]] and [[Membranous glomerulonephritis|membranous]] portion and [[stratified]] [[Columnar epithelia|columnar]] [[epithelium]] to [[stratified squamous epithelium]] in the [[Bulbous nose|bulbous]] and [[Penile discharge|penile]] portions. <br />
:* The [[submucosa]] of the [[urethra]] contains numerous glands.<br />
:* Therefore, [[Urethral cancer|urethral]] cancer in the [[male]] can manifest the histological characteristics of [[transitional cell carcinoma]], [[squamous cell carcinoma]], or [[adenocarcinoma]].<br />
:* Except for the [[prostatic urethra]], where [[transitional cell carcinoma]] is most common, [[squamous cell carcinoma]] is the predominant [[histology]] of [[urethral]] [[neoplasms]].<br />
:* [[Transitional cell carcinoma]] of the [[prostatic urethra]] may be associated with [[transitional cell carcinoma]] of the [[bladder]] and/or [[transitional cell]] [[carcinoma]] arising in [[prostatic ducts]]. Histology based on the types of the cancer:<br />
::* [[SCC]]: [[keratinization]], nuclear atypia. Positive with cytoplasmic beta-catenin stain.<br />
::* Adenocarcinoma: Columnar epithelium with [[hyperchromatic]] nuclei.<br />
::* [[Clear Cell]]: clear or eosinophilic cytoplasm, vacuoles in the cytoplasm, hyperchromatic nuclei. Positive with p53 and vimentin stain.<br />
<br />
==References==<br />
{{reflist|2}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Nephrology]]<br />
[[Category:Urologic Surgery]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Urology]]<br />
[[Category:Nephrology]]<br />
[[Category:Surgery]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_future_or_investigational_therapies&diff=1504380Adult T-cell leukemia future or investigational therapies2018-11-25T18:44:09Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
<br />
Molecular [[Target therapy|targeting therapy]] like targeting therapy against ''NOTCH1''/''FBXW7'' mutations, [[JAK|JAK-STAT]] signaling pathway and inhibition of the PI3K-signaling pathway are the future investigation therapy for adult t-cell leukemia.<ref name="pmid25966987">{{cite journal |vauthors=Litzow MR, Ferrando AA |title=How I treat T-cell acute lymphoblastic leukemia in adults |journal=Blood |volume=126 |issue=7 |pages=833–41 |date=August 2015 |pmid=25966987 |doi=10.1182/blood-2014-10-551895 |url=}}</ref><ref name="pmid25193870">{{cite journal |vauthors=Degryse S, de Bock CE, Cox L, Demeyer S, Gielen O, Mentens N, Jacobs K, Geerdens E, Gianfelici V, Hulselmans G, Fiers M, Aerts S, Meijerink JP, Tousseyn T, Cools J |title=JAK3 mutants transform hematopoietic cells through JAK1 activation, causing T-cell acute lymphoblastic leukemia in a mouse model |journal=Blood |volume=124 |issue=20 |pages=3092–100 |date=November 2014 |pmid=25193870 |doi=10.1182/blood-2014-04-566687 |url=}}</ref><ref name="pmid22516257">{{cite journal |vauthors=Subramaniam PS, Whye DW, Efimenko E, Chen J, Tosello V, De Keersmaecker K, Kashishian A, Thompson MA, Castillo M, Cordon-Cardo C, Davé UP, Ferrando A, Lannutti BJ, Diacovo TG |title=Targeting nonclassical oncogenes for therapy in T-ALL |journal=Cancer Cell |volume=21 |issue=4 |pages=459–72 |date=April 2012 |pmid=22516257 |doi=10.1016/j.ccr.2012.02.029 |url=}}</ref> <br />
<br />
{{Reflist|2}}<br />
<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Needs content]]<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_future_or_investigational_therapies&diff=1504379Adult T-cell leukemia future or investigational therapies2018-11-25T18:43:05Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
<br />
Molecular targeting therapy like targeting therapy against ''NOTCH1''/''FBXW7'' mutations, JAK-STAT signaling pathway and inhibition of the PI3K-signaling pathway are the future investigation therapy for adult t-cell leukemia.<ref name="pmid25966987">{{cite journal |vauthors=Litzow MR, Ferrando AA |title=How I treat T-cell acute lymphoblastic leukemia in adults |journal=Blood |volume=126 |issue=7 |pages=833–41 |date=August 2015 |pmid=25966987 |doi=10.1182/blood-2014-10-551895 |url=}}</ref><ref name="pmid25193870">{{cite journal |vauthors=Degryse S, de Bock CE, Cox L, Demeyer S, Gielen O, Mentens N, Jacobs K, Geerdens E, Gianfelici V, Hulselmans G, Fiers M, Aerts S, Meijerink JP, Tousseyn T, Cools J |title=JAK3 mutants transform hematopoietic cells through JAK1 activation, causing T-cell acute lymphoblastic leukemia in a mouse model |journal=Blood |volume=124 |issue=20 |pages=3092–100 |date=November 2014 |pmid=25193870 |doi=10.1182/blood-2014-04-566687 |url=}}</ref><ref name="pmid22516257">{{cite journal |vauthors=Subramaniam PS, Whye DW, Efimenko E, Chen J, Tosello V, De Keersmaecker K, Kashishian A, Thompson MA, Castillo M, Cordon-Cardo C, Davé UP, Ferrando A, Lannutti BJ, Diacovo TG |title=Targeting nonclassical oncogenes for therapy in T-ALL |journal=Cancer Cell |volume=21 |issue=4 |pages=459–72 |date=April 2012 |pmid=22516257 |doi=10.1016/j.ccr.2012.02.029 |url=}}</ref> <br />
<br />
{{Reflist|2}}<br />
<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Needs content]]<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_future_or_investigational_therapies&diff=1504378Adult T-cell leukemia future or investigational therapies2018-11-25T18:42:02Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
<br />
Molecular targeting therapy like targeting therapy against ''NOTCH1''/''FBXW7'' mutations, JAK-STAT signaling pathway and inhibition of the PI3K-signaling pathway are the future investigation therapy for adult t-cell leukemia.<ref name="pmid25193870">{{cite journal |vauthors=Degryse S, de Bock CE, Cox L, Demeyer S, Gielen O, Mentens N, Jacobs K, Geerdens E, Gianfelici V, Hulselmans G, Fiers M, Aerts S, Meijerink JP, Tousseyn T, Cools J |title=JAK3 mutants transform hematopoietic cells through JAK1 activation, causing T-cell acute lymphoblastic leukemia in a mouse model |journal=Blood |volume=124 |issue=20 |pages=3092–100 |date=November 2014 |pmid=25193870 |doi=10.1182/blood-2014-04-566687 |url=}}</ref><ref name="pmid22516257">{{cite journal |vauthors=Subramaniam PS, Whye DW, Efimenko E, Chen J, Tosello V, De Keersmaecker K, Kashishian A, Thompson MA, Castillo M, Cordon-Cardo C, Davé UP, Ferrando A, Lannutti BJ, Diacovo TG |title=Targeting nonclassical oncogenes for therapy in T-ALL |journal=Cancer Cell |volume=21 |issue=4 |pages=459–72 |date=April 2012 |pmid=22516257 |doi=10.1016/j.ccr.2012.02.029 |url=}}</ref> <br />
<br />
{{Reflist|2}}<br />
<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Needs content]]<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_future_or_investigational_therapies&diff=1504377Adult T-cell leukemia future or investigational therapies2018-11-25T18:40:52Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
<br />
Molecular targeting therapy like targeting therapy against ''NOTCH1''/''FBXW7'' mutations, JAK-STAT signaling pathway and inhibition of the PI3K-signaling pathway are the future investigation therapy for adult t-cell leukemia.<ref name="pmid25193870">{{cite journal |vauthors=Degryse S, de Bock CE, Cox L, Demeyer S, Gielen O, Mentens N, Jacobs K, Geerdens E, Gianfelici V, Hulselmans G, Fiers M, Aerts S, Meijerink JP, Tousseyn T, Cools J |title=JAK3 mutants transform hematopoietic cells through JAK1 activation, causing T-cell acute lymphoblastic leukemia in a mouse model |journal=Blood |volume=124 |issue=20 |pages=3092–100 |date=November 2014 |pmid=25193870 |doi=10.1182/blood-2014-04-566687 |url=}}</ref> <br />
<br />
{{Reflist|2}}<br />
<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Needs content]]<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_future_or_investigational_therapies&diff=1504376Adult T-cell leukemia future or investigational therapies2018-11-25T18:38:49Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
<br />
Molecular targeting therapy like targeting therapy against ''NOTCH1''/''FBXW7'' mutations, JAK-STAT signaling pathway and inhibition of the PI3K-signaling pathway are the future investigation therapy for adult t-cell leukemia. <br />
<br />
{{Reflist|2}}<br />
<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Needs content]]<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=T-cell_leukemia_natural_history,_complications_and_prognosis&diff=1502891T-cell leukemia natural history, complications and prognosis2018-11-12T23:36:09Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smouldering subtypes have a good [[prognosis]].<br />
==Natural History==<br />
* The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><br />
* Usually patients with '''acute''' adult T-cell leukemia have an aggressive clinical course with a median survival period of less than 12 months. If left untreated, most of the patients with acute adult T-cell leukemia will develop [[B symptoms|constitutional symptom]]s, [[lymphadenopathy]], and [[organomegaly]] within a few weeks of [[diagnosis]].<br />
* Usually patients with '''chronic''' adult T-cell leukemia will have an stable clinical course. If left untreated, most of the patients with chronic adult T-cell leukemia will develop [[lymphocytosis]] for months, or even years, before presenting with the typical [[cutaneous]] manifestations. <br />
* Most patients with '''smoldering''' adult T cell leukemia are initially [[asymptomatic]]. If left untreated, most of the patients with smoldering adult T cell leukemia will develop [[steroid]]-responsive [[skin]] [[rash]] and multiple [[lung]] infiltrates.<br />
<br />
==Complications==<br />
* Common complications of adult T-cell leukemia include:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><br />
:* [[Cardiac arrhythmia]]s (due to [[hypercalcaemia]])<br />
:* [[Opportunistic infection]]s ([[Strongyloides stercoralis]] infection is a frequent cause of death among adult T-cell leukemia patients)<br />
:* [[Bone]] [[fracture]]s (due to [[lytic]] [[bone]] lesions)<br />
:* [[Anemia]] <br />
:* Recurrent [[bleeding]]<br />
<br />
==Prognosis==<br />
* The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smoldering subtypes have a good [[prognosis]].<ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><br />
:* The 4-year overall survival rate of patients with acute adult T-cell leukemia is approximately 11%.<br />
:* The 4-year overall survival rate of patients with adult T-cell lumphoma is approximately 16%.<br />
:* The 4-year overall survival rate of patients with chronic adult T-cell leukemia is approximately 36%.<br />
:* The 4-year overall survival rate of patients with smouldering adult T-cell leukemia is approximately 52%.<br />
* The table below lists [[prognostic]] factors for adult T-cell leukemia patients:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378 }}</ref> <ref name="pmid29685460">{{cite journal |vauthors=Rodríguez-Zúñiga MJM, Cortez-Franco F, Qujiano-Gomero E |title=Adult T-Cell Leukemia/Lymphoma. Review of the Literature |journal=Actas Dermosifiliogr |volume=109 |issue=5 |pages=399–407 |date=June 2018 |pmid=29685460 |doi=10.1016/j.ad.2017.08.014 |url=}}</ref><br />
<br />
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"<br />
| valign="top" |<br />
|+<br />
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Prognostic Factor'''<br />
<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Clinical subtype'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Gender'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Males are associated with a worse [[prognosis]] when compared to females.<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Performance status'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Patient's poor [[performance status]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Proliferative index higher than 18%'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* is associated with a worse [[prognosis]].<br />
<br />
|-<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Calcium level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* [[Hypercalcemia]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lactate dehydrogenase (LDH) level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Elevated level of [[LDH]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''β2-microglobulin level '''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[globulin|β2-microglobulin]] level is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lymphocyte surface markers'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Over expression of [[CD25]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Neuron‐specific enolase'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[neuron]]‐specific [[enolase]] level is associated with a worse [[prognosis]].<br />
|}<br />
<br />
==References==<br />
{{reflist|2}}<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=T-cell_leukemia_natural_history,_complications_and_prognosis&diff=1502890T-cell leukemia natural history, complications and prognosis2018-11-12T23:25:54Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smouldering subtypes have a good [[prognosis]].<br />
==Natural History==<br />
* The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><br />
* Usually patients with '''acute''' adult T-cell leukemia have an aggressive clinical course with a median survival period of less than 12 months. If left untreated, most of the patients with acute adult T-cell leukemia will develop [[B symptoms|constitutional symptom]]s, [[lymphadenopathy]], and [[organomegaly]] within a few weeks of [[diagnosis]].<br />
* Usually patients with '''chronic''' adult T-cell leukemia will have an stable clinical course. If left untreated, most of the patients with chronic adult T-cell leukemia will develop [[lymphocytosis]] for months, or even years, before presenting with the typical [[cutaneous]] manifestations. <br />
* Most patients with '''smoldering''' adult T cell leukemia are initially [[asymptomatic]]. If left untreated, most of the patients with smoldering adult T cell leukemia will develop [[steroid]]-responsive [[skin]] [[rash]] and multiple [[lung]] infiltrates.<br />
<br />
==Complications==<br />
* Common complications of adult T-cell leukemia include:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><br />
:* [[Cardiac arrhythmia]]s (due to [[hypercalcaemia]])<br />
:* [[Opportunistic infection]]s ([[Strongyloides stercoralis]] infection is a frequent cause of death among adult T-cell leukemia patients)<br />
:* [[Bone]] [[fracture]]s (due to [[lytic]] [[bone]] lesions)<br />
:* [[Anemia]] <br />
:* Recurrent [[bleeding]]<br />
<br />
==Prognosis==<br />
* The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smoldering subtypes have a good [[prognosis]].<ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><br />
:* The 4-year overall survival rate of patients with acute adult T-cell leukemia is approximately 11%.<br />
:* The 4-year overall survival rate of patients with adult T-cell lumphoma is approximately 16%.<br />
:* The 4-year overall survival rate of patients with chronic adult T-cell leukemia is approximately 36%.<br />
:* The 4-year overall survival rate of patients with smouldering adult T-cell leukemia is approximately 52%.<br />
* The table below lists [[prognostic]] factors for adult T-cell leukemia patients:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378 }} </ref><br />
<br />
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"<br />
| valign="top" |<br />
|+<br />
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Prognostic Factor'''<br />
<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Clinical subtype'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Gender'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Males are associated with a worse [[prognosis]] when compared to females.<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Performance status'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Patient's poor [[performance status]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Proliferative index higher than 18%'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* is associated with a worse [[prognosis]].<br />
<br />
|-<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Calcium level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* [[Hypercalcemia]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lactate dehydrogenase (LDH) level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Elevated level of [[LDH]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''β2-microglobulin level '''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[globulin|β2-microglobulin]] level is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lymphocyte surface markers'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Over expression of [[CD25]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Neuron‐specific enolase'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[neuron]]‐specific [[enolase]] level is associated with a worse [[prognosis]].<br />
|}<br />
<br />
==References==<br />
{{reflist|2}}<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=T-cell_leukemia_natural_history,_complications_and_prognosis&diff=1502889T-cell leukemia natural history, complications and prognosis2018-11-12T23:22:45Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smouldering subtypes have a good [[prognosis]].<br />
==Natural History==<br />
* The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><br />
* Usually patients with '''acute''' adult T-cell leukemia have an aggressive clinical course with a median survival period of less than 12 months. If left untreated, most of the patients with acute adult T-cell leukemia will develop [[B symptoms|constitutional symptom]]s, [[lymphadenopathy]], and [[organomegaly]] within a few weeks of [[diagnosis]].<br />
* Usually patients with '''chronic''' adult T-cell leukemia will have an stable clinical course. If left untreated, most of the patients with chronic adult T-cell leukemia will develop [[lymphocytosis]] for months, or even years, before presenting with the typical [[cutaneous]] manifestations. <br />
* Most patients with '''smoldering''' adult T cell leukemia are initially [[asymptomatic]]. If left untreated, most of the patients with smoldering adult T cell leukemia will develop [[steroid]]-responsive [[skin]] [[rash]] and multiple [[lung]] infiltrates.<br />
<br />
==Complications==<br />
* Common complications of adult T-cell leukemia include:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><br />
:* [[Cardiac arrhythmia]]s (due to [[hypercalcaemia]])<br />
:* [[Opportunistic infection]]s ([[Strongyloides stercoralis]] infection is a frequent cause of death among adult T-cell leukemia patients)<br />
:* [[Bone]] [[fracture]]s (due to [[lytic]] [[bone]] lesions)<br />
:* [[Anemia]] <br />
:* Recurrent [[bleeding]]<br />
<br />
==Prognosis==<br />
* The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], where as chronic and smoldering subtypes have a good [[prognosis]].<ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><br />
:* The 4-year overall survival rate of patients with acute adult T-cell leukemia is approximately 11%.<br />
:* The 4-year overall survival rate of patients with adult T-cell lumphoma is approximately 16%.<br />
:* The 4-year overall survival rate of patients with chronic adult T-cell leukemia is approximately 36%.<br />
:* The 4-year overall survival rate of patients with smouldering adult T-cell leukemia is approximately 52%.<br />
* The table below lists [[prognostic]] factors for adult T-cell leukemia patients:<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794 }} </ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378 }} </ref><br />
<br />
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"<br />
| valign="top" |<br />
|+<br />
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Prognostic Factor'''<br />
<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Clinical subtype'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Gender'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Males are associated with a worse [[prognosis]] when compared to females.<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Performance status'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Patient's poor [[performance status]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Calcium level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* [[Hypercalcemia]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Proliferative index higher than 18%'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* [ is associated with a worse [[prognosis]].<br />
<br />
|-<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Absence of cutaneous lesions'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lactate dehydrogenase (LDH) level'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Elevated level of [[LDH]] is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''β2-microglobulin level '''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[globulin|β2-microglobulin]] level is associated with a worse [[prognosis]].<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Lymphocyte surface markers'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* Over expression of [[CD25]] is associated with a worse [[prognosis]].<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Neuron‐specific enolase'''|| style="padding: 5px 5px; background: #F5F5F5;" |<br />
:* An elevated [[neuron]]‐specific [[enolase]] level is associated with a worse [[prognosis]].<br />
|}<br />
<br />
==References==<br />
{{reflist|2}}<br />
{{WH}}<br />
{{WS}}<br />
<br />
[[Category:Disease]]<br />
[[Category:Pathology]]<br />
[[Category:Hematology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Oncology]]<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=User:Aida_Javanbakht&diff=1502888User:Aida Javanbakht2018-11-12T23:14:09Z<p>Aida.J: /* Pages Authored */</p>
<hr />
<div>__NOTOC__<br />
= Current Position =<br />
Associate Editor-in-Chief, PERFUSE Study Group, Beth Israel Deaconess Medical Center, Harvard Medical School.<br />
<br />
= Background and education =<br />
2008-2015: Doctor of Medicine, Mashhad University of Medical Sciences, Iran<br />
<br />
= Pages Authored =<br />
* [[Glomerular deposition disease]]<br />
* [[lupus nephritis]]<br />
* [[Hypokalemia]] <br />
* [[Hematuria]]<br />
* [[Hypernatremia]]<br />
* [[Urethral cancer]]<br />
* [[Thrombosis]]<br />
* [[Ventilation-perfusion mismatch]]<br />
* [[Cervical cancer]]<br />
* [[Amniotic fluid embolism]]<br />
* [[Adult T-cell leukemia]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502887Adult T-cell leukemia pathophysiology2018-11-12T23:12:11Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}}{{AIDA}} <br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
The exact mechanism of pathogenesis of the adult T‐cell leukemia is unknown. <br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission <ref name="pmid6261256">{{cite journal |vauthors=Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC |title=Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=77 |issue=12 |pages=7415–9 |date=December 1980 |pmid=6261256 |pmc=350514 |doi= |url=}}</ref>.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include <ref name="pmid17951212">{{cite journal |vauthors=Bittencourt AL, da Graças Vieira M, Brites CR, Farre L, Barbosa HS |title=Adult T-cell leukemia/lymphoma in Bahia, Brazil: analysis of prognostic factors in a group of 70 patients |journal=Am. J. Clin. Pathol. |volume=128 |issue=5 |pages=875–82 |date=November 2007 |pmid=17951212 |doi=10.1309/2YGD1P0QCVCWBLDX |url=}}</ref>:<br />
:* [[CD2]] +ve<br />
:* [[CD3 (immunology)|CD3]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8|CD7]] +ve<br />
:* [[CD7|CD8]] -ve<br />
:* [[CD2]]0 +ve<br />
:* [[CD25]] +ve<br />
:* [[CD79a]] +ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502886Adult T-cell leukemia classification2018-11-12T23:10:58Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}{{AIDA}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! style="background:#DCDCDC;" align="center" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| style="background:#F5F5F5;" align="left" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! style="background:#DCDCDC;" align="center" |[[Chronic adult T-cell leukemia <ref/Marker />]]<br />
| style="background:#F5F5F5;" align="left" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! style="background:#DCDCDC;" align="center" |[[Primary cutaneous tumoral (PCT)<ref/Marker />]]<br />
| style="background:#F5F5F5;" align="left" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! style="background:#DCDCDC;" align="center" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| style="background:#F5F5F5;" align="left" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! style="background:#DCDCDC;" align="center" |Adult T-cell lymphoma <ref name="pmid27925051">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref><br />
| style="background:#F5F5F5;" align="left" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia&diff=1502885Adult T-cell leukemia2018-11-12T23:09:35Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}{{AIDA}}<br />
<br />
{{SK}} T cell leukemia; T cell leukemias; T cell leukaemia; T cell leukaemias; Adult T cell leukemia; Adult T cell leukemias; Acute adult T-cell leukemia; Chronic adult T-cell leukemia; Smouldering adult T-cell leukemia; Adult T-cell lymphoma<br />
<br />
==[[Adult T-cell leukemia overview|Overview]]==<br />
<br />
==[[Adult T-cell leukemia historical perspective|Historical Perspective]]==<br />
<br />
==[[Adult T-cell leukemia classification|Classification]]==<br />
<br />
==[[Adult T-cell leukemia pathophysiology|Pathophysiology]]==<br />
<br />
==[[Adult T-cell leukemia causes|Causes]]==<br />
<br />
==[[Adult T-cell leukemia differential diagnosis|Differentiating Adult T-cell leukemia from Other Diseases]]==<br />
<br />
==[[Adult T-cell leukemia epidemiology and demographics|Epidemiology and Demographics]]==<br />
<br />
==[[Adult T-cell leukemia risk factors|Risk Factors]]==<br />
<br />
==[[Adult T-cell leukemia screening|Screening]]==<br />
<br />
==[[T-cell leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==<br />
<br />
==Diagnosis==<br />
[[Adult T-cell leukemia diagnostic study of choice|Diagnostic Study of Choice]] | [[Adult T-cell leukemia history and symptoms|History and Symptoms]] | [[Adult T-cell leukemia physical examination|Physical Examination]] | [[Adult T-cell leukemia laboratory findings|Laboratory Findings]] | [[Adult T-cell leukemia electrocardiogram|Electrocardiogram]] | [[Adult T-cell leukemia x ray|X-Ray Findings]] | [[Adult T-cell leukemia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Adult T-cell leukemia CT|CT scan]] | [[Adult T-cell leukemia MRI|MRI Findings]] | [[Adult T-cell leukemia other imaging findings|Other Imaging Findings]] | [[Adult T-cell leukemia other diagnostic studies|Other Diagnostic Studies]]<br />
<br />
==Treatment==<br />
[[Adult T-cell leukemia medical therapy|Medical Therapy]] | [[Adult T-cell leukemia interventions|Interventions]] | [[Adult T-cell leukemia surgery|Surgery]] | [[Adult T-cell leukemia primary prevention|Primary Prevention]] | [[Adult T-cell leukemia secondary prevention|Secondary Prevention]] | [[Adult T-cell leukemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Adult T-cell leukemia future or investigational therapies|Future or Investigational Therapies]]<br />
<br />
==Case Studies==<br />
[[Adult T-cell leukemia case study one|Case #1]]<br />
<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_differential_diagnosis&diff=1502884Adult T-cell leukemia differential diagnosis2018-11-12T23:06:52Z<p>Aida.J: /* Differentiating Adult T-cell Leukemia from other Diseases */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
<br />
==Overview==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Differentiating Adult T-cell Leukemia from other Diseases==<br />
* Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid20198459">{{cite journal |vauthors=Chuang SS, Ichinohasama R, Chu JS, Ohshima K |title=Differential diagnosis of angioimmunoblastic T-cell lymphoma with seropositivity for anti-HTLV antibody from adult T-cell leukemia/lymphoma |journal=Int. J. Hematol. |volume=91 |issue=4 |pages=687–91 |date=May 2010 |pmid=20198459 |doi=10.1007/s12185-010-0540-x |url=}}</ref><ref name="H">Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011</ref><ref name="pmid29685460">{{cite journal |vauthors=Rodríguez-Zúñiga MJM, Cortez-Franco F, Qujiano-Gomero E |title=Adult T-Cell Leukemia/Lymphoma. Review of the Literature |journal=Actas Dermosifiliogr |volume=109 |issue=5 |pages=399–407 |date=June 2018 |pmid=29685460 |doi=10.1016/j.ad.2017.08.014 |url=}}</ref><br />
* Based on the expression of cell surface markers, the table below differentiates adult T-cell leukemia from other diseases that cause similar clinical presentations:<br />
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"<br />
<br />
|+<br />
! style="background: #4479BA; width: 600px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Surface Immunoglobulin'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD5'''}}<br />
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|'''CD22/FMC7'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD23'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD79b'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD103'''}}<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Chronic lymphocytic leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Weakly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive/Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Prolymphocytic leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Hairy cell leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive/Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Mantle cell lymphoma'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Follicular lymphoma'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative <br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
|}<br />
<br><br />
* Adult T-cell leukemia must also be differentiated from other causes of [[fever]], [[hepatosplenomegaly]], and [[lymph node]] swelling such as:<br />
:* [[Splenic marginal zone lymphoma]]<br />
:* Nodal marginal zone [[lymphoma]]<br />
:* [[Lymphoplasmacytic lymphoma]]<br />
:* [[Sézary syndrome]]<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_differential_diagnosis&diff=1502883Adult T-cell leukemia differential diagnosis2018-11-12T23:04:32Z<p>Aida.J: /* Differentiating Adult T-cell Leukemia from other Diseases */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
<br />
==Overview==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Differentiating Adult T-cell Leukemia from other Diseases==<br />
* Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid20198459">{{cite journal |vauthors=Chuang SS, Ichinohasama R, Chu JS, Ohshima K |title=Differential diagnosis of angioimmunoblastic T-cell lymphoma with seropositivity for anti-HTLV antibody from adult T-cell leukemia/lymphoma |journal=Int. J. Hematol. |volume=91 |issue=4 |pages=687–91 |date=May 2010 |pmid=20198459 |doi=10.1007/s12185-010-0540-x |url=}}</ref><ref name="H">Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011</ref><br />
* Based on the expression of cell surface markers, the table below differentiates adult T-cell leukemia from other diseases that cause similar clinical presentations:<br />
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"<br />
<br />
|+<br />
! style="background: #4479BA; width: 600px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Surface Immunoglobulin'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD5'''}}<br />
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|'''CD22/FMC7'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD23'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD79b'''}}<br />
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''CD103'''}}<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Chronic lymphocytic leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Weakly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive/Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Prolymphocytic leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Hairy cell leukemia'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive/Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Mantle cell lymphoma'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
<br />
|-<br />
<br />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |<br />
'''Follicular lymphoma'''<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative <br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Strongly positive<br />
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |<br />
Negative<br />
|}<br />
<br><br />
* Adult T-cell leukemia must also be differentiated from other causes of [[fever]], [[hepatosplenomegaly]], and [[lymph node]] swelling such as:<br />
:* [[Splenic marginal zone lymphoma]]<br />
:* Nodal marginal zone [[lymphoma]]<br />
:* [[Lymphoplasmacytic lymphoma]]<br />
:* [[Sézary syndrome]]<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502881Adult T-cell leukemia pathophysiology2018-11-12T23:00:31Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}} <br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
The exact mechanism of pathogenesis of the adult T‐cell leukemia is unknown. <br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission <ref name="pmid6261256">{{cite journal |vauthors=Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC |title=Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=77 |issue=12 |pages=7415–9 |date=December 1980 |pmid=6261256 |pmc=350514 |doi= |url=}}</ref>.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include <ref name="pmid17951212">{{cite journal |vauthors=Bittencourt AL, da Graças Vieira M, Brites CR, Farre L, Barbosa HS |title=Adult T-cell leukemia/lymphoma in Bahia, Brazil: analysis of prognostic factors in a group of 70 patients |journal=Am. J. Clin. Pathol. |volume=128 |issue=5 |pages=875–82 |date=November 2007 |pmid=17951212 |doi=10.1309/2YGD1P0QCVCWBLDX |url=}}</ref>:<br />
:* [[CD2]] +ve<br />
:* [[CD3 (immunology)|CD3]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8|CD7]] +ve<br />
:* [[CD7|CD8]] -ve<br />
:* [[CD2]]0 +ve<br />
:* [[CD25]] +ve<br />
:* [[CD79a]] +ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502878Adult T-cell leukemia pathophysiology2018-11-12T22:59:31Z<p>Aida.J: /* Microscopic Pathology */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
The exact mechanism of pathogenesis of the adult T‐cell leukemia is unknown. <br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission <ref name="pmid6261256">{{cite journal |vauthors=Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC |title=Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=77 |issue=12 |pages=7415–9 |date=December 1980 |pmid=6261256 |pmc=350514 |doi= |url=}}</ref>.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include <ref name="pmid17951212">{{cite journal |vauthors=Bittencourt AL, da Graças Vieira M, Brites CR, Farre L, Barbosa HS |title=Adult T-cell leukemia/lymphoma in Bahia, Brazil: analysis of prognostic factors in a group of 70 patients |journal=Am. J. Clin. Pathol. |volume=128 |issue=5 |pages=875–82 |date=November 2007 |pmid=17951212 |doi=10.1309/2YGD1P0QCVCWBLDX |url=}}</ref>:<br />
:* [[CD2]] +ve<br />
:* [[CD3 (immunology)|CD3]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8|CD7]] +ve<br />
:* [[CD7|CD8]] -ve<br />
:* [[CD2]]0 +ve<br />
:* [[CD25]] +ve<br />
:* [[CD79a]] +ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502872Adult T-cell leukemia classification2018-11-12T22:42:21Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia <ref/Marker />]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)<ref/Marker />]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma <ref name="pmid27925051">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502871Adult T-cell leukemia classification2018-11-12T22:39:01Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia <ref name="pmid10659498">{{cite journal |vauthors=Setoyama M, Katahira Y, Kanzaki T |title=Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought |journal=J. Dermatol. |volume=26 |issue=12 |pages=785–90 |date=December 1999 |pmid=10659498 |doi= |url=}}</ref>]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)<ref name="pmid22830614">{{cite journal |vauthors=Sawada Y, Shimauchi T, Yamaguchi T, Okura R, Hama-Yamamoto K, Fueki-Yoshioka H, Ohmori S, Yamada S, Yoshizawa M, Hiromasa K, Tajiri M, Kabashima-Kubo R, Yoshioka M, Sugita K, Yoshiki R, Hino R, Kobayashi M, Izu K, Nakamura M, Tokura Y |title=Combination of skin-directed therapy and oral etoposide for smoldering adult T-cell leukemia/lymphoma with skin involvement |journal=Leuk. Lymphoma |volume=54 |issue=3 |pages=520–7 |date=March 2013 |pmid=22830614 |doi=10.3109/10428194.2012.715351 |url=}}</ref>]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma <ref name="pmid27925051">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502870Adult T-cell leukemia classification2018-11-12T22:37:45Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia <ref name="pmid10659498">{{cite journal |vauthors=Setoyama M, Katahira Y, Kanzaki T |title=Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought |journal=J. Dermatol. |volume=26 |issue=12 |pages=785–90 |date=December 1999 |pmid=10659498 |doi= |url=}}</ref>]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)<ref name="pmid22830614">{{cite journal |vauthors=Sawada Y, Shimauchi T, Yamaguchi T, Okura R, Hama-Yamamoto K, Fueki-Yoshioka H, Ohmori S, Yamada S, Yoshizawa M, Hiromasa K, Tajiri M, Kabashima-Kubo R, Yoshioka M, Sugita K, Yoshiki R, Hino R, Kobayashi M, Izu K, Nakamura M, Tokura Y |title=Combination of skin-directed therapy and oral etoposide for smoldering adult T-cell leukemia/lymphoma with skin involvement |journal=Leuk. Lymphoma |volume=54 |issue=3 |pages=520–7 |date=March 2013 |pmid=22830614 |doi=10.3109/10428194.2012.715351 |url=}}</ref>]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502869Adult T-cell leukemia classification2018-11-12T22:35:45Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia <ref name="pmid10659498">{{cite journal |vauthors=Setoyama M, Katahira Y, Kanzaki T |title=Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought |journal=J. Dermatol. |volume=26 |issue=12 |pages=785–90 |date=December 1999 |pmid=10659498 |doi= |url=}}</ref>]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502868Adult T-cell leukemia classification2018-11-12T22:34:01Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia <ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |date=December 2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502867Adult T-cell leukemia classification2018-11-12T22:30:37Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<ref name="pmid19064971">{{cite journal |vauthors=Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, O'Mahony D, Janik JE, Bittencourt AL, Taylor GP, Yamaguchi K, Utsunomiya A, Tobinai K, Watanabe T |title=Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting |journal=J. Clin. Oncol. |volume=27 |issue=3 |pages=453–9 |date=January 2009 |pmid=19064971 |pmc=2737379 |doi=10.1200/JCO.2008.18.2428 |url=}}</ref><br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_classification&diff=1502866Adult T-cell leukemia classification2018-11-12T22:27:57Z<p>Aida.J: </p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}} <br />
<br />
==Overview==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Classification==<br />
* Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693 }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370 }} </ref><br />
* The table below lists the adult T-cell leukemia clinical classification details:<br />
<br />
{| <br />
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |Clinical Variant<br />
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |Description<br />
|-<br />
! align="center" style="background:#DCDCDC;" |Acute adult T-cell leukemia<br />
| align="left" style="background:#F5F5F5;" | <br />
* Leukemic clinical presentation <br />
* [[Organomegaly]] present <br />
* Markedly elevated [[lactate dehydrogenase]] ([[LDH]]) level<br />
* [[Hypercalcaemia]]<br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Chronic adult T-cell leukemia]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Lymphocyte]] count greater than 4×10<sup>9</sup>/L <br />
* [[Cutaneous]], [[pulmonary]], and [[hepatic]] involvement <br />
* [[Lymphadenopathy]] <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |[[Primary cutaneous tumoral (PCT)]]<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] involvement. Nodules on the skin. <br />
* Poor prognosis. <br />
* Normal [[serum]] [[LDH]] level (or less that twice the normal upper limit)<br />
* Normal [[serum]] [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Smoldering adult T-cell leukemia<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Cutaneous]] and/or [[pulmonary]] infiltrates<br />
* No other organ involvement<br />
* Normal [[lymphocyte]] count (1–5% ATLL cells)<br />
* Normal serum [[Lactate dehydrogenase|LDH]] level<br />
* Normal serum [[calcium]] level <br />
|-<br />
! align="center" style="background:#DCDCDC;" |Adult T-cell lymphoma<br />
| align="left" style="background:#F5F5F5;" | <br />
* [[Organomegaly]] present<br />
* Less than 1% leukemic cells in the [[circulation]]<br />
* Elevated [[serum]] [[LDH]] level <br />
* [[Hypercalcemia]] <br />
|}<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502862Adult T-cell leukemia pathophysiology2018-11-12T20:37:03Z<p>Aida.J: /* Pathogenesis */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
The exact mechanism of pathogenesis of the adult T‐cell leukemia is unknown. <br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission <ref name="pmid6261256">{{cite journal |vauthors=Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC |title=Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=77 |issue=12 |pages=7415–9 |date=December 1980 |pmid=6261256 |pmc=350514 |doi= |url=}}</ref>.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include:<br />
:* [[CD2]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8]] +ve<br />
:* [[CD7]] -ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_overview&diff=1502860Adult T-cell leukemia overview2018-11-12T20:28:35Z<p>Aida.J: /* Epidemiology and Demographics */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant<ref name="pmid30407885">{{cite journal |vauthors=Guru Murthy GS, Pondaiah SK, Abedin S, Atallah E |title=Incidence and survival of T-cell acute lymphoblastic leukemia in the United States |journal=Leuk. Lymphoma |volume= |issue= |pages=1–8 |date=November 2018 |pmid=30407885 |doi=10.1080/10428194.2018.1522442 |url=}}</ref><ref name="pmid28554708">{{cite journal |vauthors=Fernández MS, Rojas FD, Cattana ME, Mussin JE, de Los Ángeles Sosa M, Benzoni CD, Giusiano GE |title=Protothecosis in a patient with T cell lymphocytic leukemia |journal=Rev. Argent. Microbiol. |volume=49 |issue=3 |pages=224–226 |date=2017 |pmid=28554708 |doi=10.1016/j.ram.2017.02.007 |url=}}</ref>.<br />
<br />
==Historical Perspective==<br />
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981. <ref name="pmid27979862">{{cite journal |vauthors= |title=Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood. 1977;50(3):481-492 |journal=Blood |volume=128 |issue=24 |pages=2745 |date=December 2016 |pmid=27979862 |doi=10.1182/blood-2016-10-746677 |url=}}</ref><br />
<br />
==Classification==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Pathophysiology==<br />
Adult T‐cell leukemia arises from post‐thymic [[Lymphocyte|lymphocytes]], which are normally involved in the process of [[Immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[Genetic mutation|genetic mutations]] induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid28202003">{{cite journal |vauthors=Santos RF, Conceição GC, Martins MS, Kraychete A, Penalva MA, Carvalho EM, Lopes AA, Rocha PN |title=Prevalence and risk factors for Human T-Lymphotropic Virus Type 1 (HTLV-1) among maintenance hemodialysis patients |journal=BMC Nephrol |volume=18 |issue=1 |pages=64 |date=February 2017 |pmid=28202003 |pmc=5312583 |doi=10.1186/s12882-017-0484-y |url=}}</ref>. On gross pathology, skin [[Nodule|nodules]], [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Causes==<br />
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[Genetic mutation|genetic mutations]] involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].<br />
<br />
==Differentiating Adult T-cell leukemia from other Diseases==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[Lymph node|lymph nodes]], such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Epidemiology and Demographics==<br />
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia <ref name="pmid30089749">{{cite journal |vauthors=Kawano N, Yoshida S, Kawano S, Kuriyama T, Tahara Y, Toyofuku A, Manabe T, Doi A, Terasaka S, Yamashita K, Ueda Y, Ochiai H, Marutsuka K, Yamano Y, Shimoda K, Kikuchi I |title=The clinical impact of human T-lymphotrophic virus type 1 (HTLV-1) infection on the development of adult T-cell leukemia-lymphoma (ATL) or HTLV-1-associated myelopathy (HAM) / atypical HAM after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and renal transplantation |journal=J Clin Exp Hematop |volume=58 |issue=3 |pages=107–121 |date=September 2018 |pmid=30089749 |doi=10.3960/jslrt.18011 |url=}}</ref> <ref name="pmid22973265">{{cite journal |vauthors=Iwanaga M, Watanabe T, Yamaguchi K |title=Adult T-cell leukemia: a review of epidemiological evidence |journal=Front Microbiol |volume=3 |issue= |pages=322 |date=2012 |pmid=22973265 |pmc=3437524 |doi=10.3389/fmicb.2012.00322 |url=}}</ref>.<br />
<br />
==Risk Factors==<br />
Common [[risk factors]] in the development of adult T-cell leukemia among [[HTLV]] carriers are [[vertical transmission]] of HTLV [[infection]] during [[Childbirth|labor]], male sex, and specific [[Human leukocyte antigen|human leukocyte antigens]] such as [[HLA-A]] 26, [[HLA-B]] 4002, and [[HLA-B]] 4801. <br />
<br />
==Screening==<br />
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for adult T-cell leukemia.<br />
<br />
==Natural History, Complications and Prognosis==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[Cardiac arrhythmia|cardiac arrhythmias]], [[Opportunistic infection|opportunistic infections]] , and [[Bone fracture|bone fractures]]. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], whereas chronic and smouldering subtypes have a good [[prognosis]].<br />
<br />
==Diagnosis==<br />
===Diagnostic Study of Choice===<br />
There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic [[histopathology]] and [[Human T-lymphotropic virus|HTLV]]-1 infection.<br />
<br />
===History and Symptoms===<br />
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and recurrent [[infection]]s.<br />
<br />
===Physical Examination===<br />
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].<br />
<br />
===Laboratory Findings===<br />
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.<br />
<br />
===Electrocardiogram===<br />
There are no ECG findings associated with adult T-cell leukemia. However, [[electrocardiogram]] might be helpful in detecting complications of adult T-cell leukemia such as [[Cardiac arrhythmia|cardiac arrhythmias]] due to [[hypercalcemia]]. To view the electrocardiogram findings in [[hypercalcemia]], click [[Hypercalcemia electrocardiogram|here]].<br />
<br />
===X-ray===<br />
There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia which include [[bone fractures]] and lytic lesions.<br />
<br />
=== Echocardiography and Ultrasound ===<br />
There are no [[echocardiography]] findings associated with adult T-cell leukemia. Findings on an [[ultrasound]] suggestive of adult T-cell leukemia include [[hepatomegaly]], [[splenomegaly]] and [[lymphadenopathy]].<br />
<br />
===CT Scan===<br />
[[Thoracic]] [[CT scan]] may be helpful in the diagnosis of adult T-cell leukemia. Findings on [[CT scan]] suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations. Findings on abdominal [[CT scan]] suggestive of adult T-cell leukemia cells include [[hepatomegaly]] and [[splenomegaly]].<br />
<br />
===MRI===<br />
There are no MRI findings associated with adult T-cell leukemia.<br />
<br />
=== Other Imaging Findings ===<br />
There are no other imaging findings associated with adult T-cell leukemia.<br />
<br />
===Other Diagnostic Studies===<br />
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization]]'''.'''<br />
<br />
==Treatment==<br />
===Medical Therapy===<br />
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell transplant]], or a combination of [[zidovudine]] and [[interferon]] therapy. While adult T-cell lymphoma patients are usually managed by either [[chemotherapy]], supportive care, or allogeneic [[stem cell]] transplant.<br />
<br />
===Interventions===<br />
There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.<br />
<br />
===Surgery===<br />
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.<br />
<br />
=== Primary Prevention ===<br />
Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of [[Human T-lymphotropic virus|HTLV]] virus. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.<br />
<br />
=== Secondary Prevention ===<br />
There are no established measures for the secondary prevention of Adult T-cell leukemia.<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502859Adult T-cell leukemia pathophysiology2018-11-12T20:25:02Z<p>Aida.J: /* Pathogenesis */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission <ref name="pmid6261256">{{cite journal |vauthors=Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC |title=Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=77 |issue=12 |pages=7415–9 |date=December 1980 |pmid=6261256 |pmc=350514 |doi= |url=}}</ref>.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include:<br />
:* [[CD2]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8]] +ve<br />
:* [[CD7]] -ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_pathophysiology&diff=1502858Adult T-cell leukemia pathophysiology2018-11-12T20:23:43Z<p>Aida.J: /* Genetic */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}}{{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Pathogenesis==<br />
* Adult T‐cell leukemia arises from post‐thymic [[lymphocytes]], which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
* Adult T‐cell leukemia is mainly caused by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid279250512">{{cite journal |vauthors=Oliveira PD, Farre L, Bittencourt AL |title=Adult T-cell leukemia/lymphoma |journal=Rev Assoc Med Bras (1992) |volume=62 |issue=7 |pages=691–700 |date=October 2016 |pmid=27925051 |doi=10.1590/1806-9282.62.07.691 |url=}}</ref>.<br />
* [[HTLV]] is usually transmitted via [[breast feeding]] early in life. Other minor routes of transmission for [[Human T-lymphotropic virus|HTLV]] may include [[sexual contact]], exposure to [[contamination|contaminated]] [[blood]], or vertical maternal transmission.<br />
* There appears to be a long latent period between [[Human T-lymphotropic virus|HTLV]]-1 infection and the development of adult T‐cell leukemia.<br />
* The oncogenesis of [[Human T-lymphotropic virus|HTLV]] infection, which results in the development of adult T-cell leukemia, is due to:<br />
:* [[Human T-lymphotropic virus|HTLV]] basic [[leucine zipper]] factor <br />
:* [[Human T-lymphotropic virus|HTLV]] p40 tax viral [[protein]]<br />
:* Activation of [[JAK]]/[[STAT]] signaling pathway by [[Human T-lymphotropic virus|HTLV]]<br />
:* Enhancement of [[CREB]] [[transcription factor]] by [[Human T-lymphotropic virus|HTLV]]<br />
* Adult T‐cell leukemia can manifests as either a [[leukemic]] form (75% of the cases) or a pure [[lymphoma|lymphomatous]] form (25% of the cases). <br />
* Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral [[Blood cell|blood cells]], [[bone marrow]], [[lymph node]]s, [[liver]], [[spleen]], [[skin]], and [[CNS]].<br />
* Hematopathological features of adult T-cell leukemia are variable, which may include:<br />
:* [[Anemia]]<br />
:* [[Thrombocytopenia]]<br />
:* [[Neutrophilia]]<br />
:* [[Eosinophilia]]<br />
* Patchy [[Bone marrow suppression|bone marrow infiltration]] among adult T-cell leukemia patients may result in:<br />
:* Tumor-induced [[osteolysis]] due to increased [[osteoclast|osteoclastic activity]]<br />
:* Multiple [[lytic]] bone lesions<br />
:* [[Hypercalcemia]]<br />
* [[Hypercalcemia]] among adult T-cell leukemia patients has been associated with elevated serum concentrations of:<br />
:* [[IL-1]]<br />
:* [[TGFβ]]<br />
:* [[PTHrP]]<br />
:* MIP-1α<br />
:* [[RANKL]]<br />
* Infiltration of malignant leukemic cells results in the expansion of the [[lymph node]]s paracortical region, which may lead to the development of peripheral [[lymphadenopathy]] among adult T-cell leukemia patients.<br />
* Infiltration of the [[liver]] and [[spleen]] may lead to the development of [[organomegaly]] among adult T-cell leukemia patients.<br />
* [[Cutaneous]] manifestations of adult T-cell leukemia is due to the infiltration of [[Leukemic|leukemic cells]] along the [[dermis]] layer of the [[skin]].<br />
* Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in [[Sézary syndrome]] and [[mycosis fungoides]]. <br />
* [[Immune deficiency]] occurs in adult T-cell leukemia due to a defective [[cell-mediated immunity]].<br />
<br />
==Genetic==<br />
* Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s.<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><br />
<br />
* [[Gene]]s involved in the pathogenesis of adult T-cell leukemia include: <br />
** 14q11 gene mutation<br />
** [[TCR|TCR‐alpha]] chain gene mutation <br />
** [[TCR|TCR‐delta]] chain gene mutation<br />
==Gross Pathology==<br />
* On gross pathology, [[skin]] nodules, [[maculopapular]] eruption, and [[erythema]] are characteristic findings of adult T-cell leukemia.<ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref><ref name="pmid18377598">{{cite journal| author=Pezeshkpoor F, Yazdanpanah MJ, Shirdel A| title=Specific cutaneous manifestations in adult T-cell leukemia/lymphoma. | journal=Int J Dermatol | year= 2008 | volume= 47 | issue= 4 | pages= 359-62 | pmid=18377598 | doi=10.1111/j.1365-4632.2008.03526.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377598 }} </ref><br />
<br />
==Microscopic Pathology==<br />
* On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:<br />
:* Pleomorphic, medium sized [[lymphocyte]]s<br />
:* Convoluted or polylobulated [[nucleus]] with condensed [[chromatin]] (cloverleaf nuclei)<br />
:* [[Nucleoli]] are not visible<br />
:* Agranular [[cytoplasm]] <br />
:* “Flower cells”<br />
:* [[Reed-Sternberg cells|Reed-Sternberg]]-like cells may also be present<br />
<br />
* On [[Immunohistochemistry|immunohistochemical]] analysis, characteristic findings of adult T-cell leukemia include:<br />
:* [[CD2]] +ve<br />
:* [[CD4]] +ve<br />
:* [[CD5]] +ve<br />
:* [[CD8]] +ve<br />
:* [[CD7]] -ve<br />
:* CD3 and [[TCR|T‐cell receptor]] (TCR)‐β may be down‐regulated<br />
<br />
==References==<br />
{{reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_overview&diff=1502855Adult T-cell leukemia overview2018-11-12T20:10:46Z<p>Aida.J: /* Epidemiology and Demographics */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant<ref name="pmid30407885">{{cite journal |vauthors=Guru Murthy GS, Pondaiah SK, Abedin S, Atallah E |title=Incidence and survival of T-cell acute lymphoblastic leukemia in the United States |journal=Leuk. Lymphoma |volume= |issue= |pages=1–8 |date=November 2018 |pmid=30407885 |doi=10.1080/10428194.2018.1522442 |url=}}</ref><ref name="pmid28554708">{{cite journal |vauthors=Fernández MS, Rojas FD, Cattana ME, Mussin JE, de Los Ángeles Sosa M, Benzoni CD, Giusiano GE |title=Protothecosis in a patient with T cell lymphocytic leukemia |journal=Rev. Argent. Microbiol. |volume=49 |issue=3 |pages=224–226 |date=2017 |pmid=28554708 |doi=10.1016/j.ram.2017.02.007 |url=}}</ref>.<br />
<br />
==Historical Perspective==<br />
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981. <ref name="pmid27979862">{{cite journal |vauthors= |title=Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood. 1977;50(3):481-492 |journal=Blood |volume=128 |issue=24 |pages=2745 |date=December 2016 |pmid=27979862 |doi=10.1182/blood-2016-10-746677 |url=}}</ref><br />
<br />
==Classification==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Pathophysiology==<br />
Adult T‐cell leukemia arises from post‐thymic [[Lymphocyte|lymphocytes]], which are normally involved in the process of [[Immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[Genetic mutation|genetic mutations]] induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid28202003">{{cite journal |vauthors=Santos RF, Conceição GC, Martins MS, Kraychete A, Penalva MA, Carvalho EM, Lopes AA, Rocha PN |title=Prevalence and risk factors for Human T-Lymphotropic Virus Type 1 (HTLV-1) among maintenance hemodialysis patients |journal=BMC Nephrol |volume=18 |issue=1 |pages=64 |date=February 2017 |pmid=28202003 |pmc=5312583 |doi=10.1186/s12882-017-0484-y |url=}}</ref>. On gross pathology, skin [[Nodule|nodules]], [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Causes==<br />
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[Genetic mutation|genetic mutations]] involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].<br />
<br />
==Differentiating Adult T-cell leukemia from other Diseases==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[Lymph node|lymph nodes]], such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Epidemiology and Demographics==<br />
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia <ref name="pmid30089749">{{cite journal |vauthors=Kawano N, Yoshida S, Kawano S, Kuriyama T, Tahara Y, Toyofuku A, Manabe T, Doi A, Terasaka S, Yamashita K, Ueda Y, Ochiai H, Marutsuka K, Yamano Y, Shimoda K, Kikuchi I |title=The clinical impact of human T-lymphotrophic virus type 1 (HTLV-1) infection on the development of adult T-cell leukemia-lymphoma (ATL) or HTLV-1-associated myelopathy (HAM) / atypical HAM after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and renal transplantation |journal=J Clin Exp Hematop |volume=58 |issue=3 |pages=107–121 |date=September 2018 |pmid=30089749 |doi=10.3960/jslrt.18011 |url=}}</ref>.<br />
<br />
==Risk Factors==<br />
Common [[risk factors]] in the development of adult T-cell leukemia among [[HTLV]] carriers are [[vertical transmission]] of HTLV [[infection]] during [[Childbirth|labor]], male sex, and specific [[Human leukocyte antigen|human leukocyte antigens]] such as [[HLA-A]] 26, [[HLA-B]] 4002, and [[HLA-B]] 4801. <br />
<br />
==Screening==<br />
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for adult T-cell leukemia.<br />
<br />
==Natural History, Complications and Prognosis==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[Cardiac arrhythmia|cardiac arrhythmias]], [[Opportunistic infection|opportunistic infections]] , and [[Bone fracture|bone fractures]]. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], whereas chronic and smouldering subtypes have a good [[prognosis]].<br />
<br />
==Diagnosis==<br />
===Diagnostic Study of Choice===<br />
There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic [[histopathology]] and [[Human T-lymphotropic virus|HTLV]]-1 infection.<br />
<br />
===History and Symptoms===<br />
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and recurrent [[infection]]s.<br />
<br />
===Physical Examination===<br />
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].<br />
<br />
===Laboratory Findings===<br />
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.<br />
<br />
===Electrocardiogram===<br />
There are no ECG findings associated with adult T-cell leukemia. However, [[electrocardiogram]] might be helpful in detecting complications of adult T-cell leukemia such as [[Cardiac arrhythmia|cardiac arrhythmias]] due to [[hypercalcemia]]. To view the electrocardiogram findings in [[hypercalcemia]], click [[Hypercalcemia electrocardiogram|here]].<br />
<br />
===X-ray===<br />
There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia which include [[bone fractures]] and lytic lesions.<br />
<br />
=== Echocardiography and Ultrasound ===<br />
There are no [[echocardiography]] findings associated with adult T-cell leukemia. Findings on an [[ultrasound]] suggestive of adult T-cell leukemia include [[hepatomegaly]], [[splenomegaly]] and [[lymphadenopathy]].<br />
<br />
===CT Scan===<br />
[[Thoracic]] [[CT scan]] may be helpful in the diagnosis of adult T-cell leukemia. Findings on [[CT scan]] suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations. Findings on abdominal [[CT scan]] suggestive of adult T-cell leukemia cells include [[hepatomegaly]] and [[splenomegaly]].<br />
<br />
===MRI===<br />
There are no MRI findings associated with adult T-cell leukemia.<br />
<br />
=== Other Imaging Findings ===<br />
There are no other imaging findings associated with adult T-cell leukemia.<br />
<br />
===Other Diagnostic Studies===<br />
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization]]'''.'''<br />
<br />
==Treatment==<br />
===Medical Therapy===<br />
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell transplant]], or a combination of [[zidovudine]] and [[interferon]] therapy. While adult T-cell lymphoma patients are usually managed by either [[chemotherapy]], supportive care, or allogeneic [[stem cell]] transplant.<br />
<br />
===Interventions===<br />
There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.<br />
<br />
===Surgery===<br />
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.<br />
<br />
=== Primary Prevention ===<br />
Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of [[Human T-lymphotropic virus|HTLV]] virus. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.<br />
<br />
=== Secondary Prevention ===<br />
There are no established measures for the secondary prevention of Adult T-cell leukemia.<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_overview&diff=1502854Adult T-cell leukemia overview2018-11-12T20:08:38Z<p>Aida.J: /* Pathophysiology */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant<ref name="pmid30407885">{{cite journal |vauthors=Guru Murthy GS, Pondaiah SK, Abedin S, Atallah E |title=Incidence and survival of T-cell acute lymphoblastic leukemia in the United States |journal=Leuk. Lymphoma |volume= |issue= |pages=1–8 |date=November 2018 |pmid=30407885 |doi=10.1080/10428194.2018.1522442 |url=}}</ref><ref name="pmid28554708">{{cite journal |vauthors=Fernández MS, Rojas FD, Cattana ME, Mussin JE, de Los Ángeles Sosa M, Benzoni CD, Giusiano GE |title=Protothecosis in a patient with T cell lymphocytic leukemia |journal=Rev. Argent. Microbiol. |volume=49 |issue=3 |pages=224–226 |date=2017 |pmid=28554708 |doi=10.1016/j.ram.2017.02.007 |url=}}</ref>.<br />
<br />
==Historical Perspective==<br />
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981. <ref name="pmid27979862">{{cite journal |vauthors= |title=Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood. 1977;50(3):481-492 |journal=Blood |volume=128 |issue=24 |pages=2745 |date=December 2016 |pmid=27979862 |doi=10.1182/blood-2016-10-746677 |url=}}</ref><br />
<br />
==Classification==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Pathophysiology==<br />
Adult T‐cell leukemia arises from post‐thymic [[Lymphocyte|lymphocytes]], which are normally involved in the process of [[Immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[Genetic mutation|genetic mutations]] induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]) <ref name="pmid28202003">{{cite journal |vauthors=Santos RF, Conceição GC, Martins MS, Kraychete A, Penalva MA, Carvalho EM, Lopes AA, Rocha PN |title=Prevalence and risk factors for Human T-Lymphotropic Virus Type 1 (HTLV-1) among maintenance hemodialysis patients |journal=BMC Nephrol |volume=18 |issue=1 |pages=64 |date=February 2017 |pmid=28202003 |pmc=5312583 |doi=10.1186/s12882-017-0484-y |url=}}</ref>. On gross pathology, skin [[Nodule|nodules]], [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Causes==<br />
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[Genetic mutation|genetic mutations]] involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].<br />
<br />
==Differentiating Adult T-cell leukemia from other Diseases==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[Lymph node|lymph nodes]], such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Epidemiology and Demographics==<br />
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.<br />
<br />
==Risk Factors==<br />
Common [[risk factors]] in the development of adult T-cell leukemia among [[HTLV]] carriers are [[vertical transmission]] of HTLV [[infection]] during [[Childbirth|labor]], male sex, and specific [[Human leukocyte antigen|human leukocyte antigens]] such as [[HLA-A]] 26, [[HLA-B]] 4002, and [[HLA-B]] 4801. <br />
<br />
==Screening==<br />
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for adult T-cell leukemia.<br />
<br />
==Natural History, Complications and Prognosis==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[Cardiac arrhythmia|cardiac arrhythmias]], [[Opportunistic infection|opportunistic infections]] , and [[Bone fracture|bone fractures]]. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], whereas chronic and smouldering subtypes have a good [[prognosis]].<br />
<br />
==Diagnosis==<br />
===Diagnostic Study of Choice===<br />
There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic [[histopathology]] and [[Human T-lymphotropic virus|HTLV]]-1 infection.<br />
<br />
===History and Symptoms===<br />
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and recurrent [[infection]]s.<br />
<br />
===Physical Examination===<br />
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].<br />
<br />
===Laboratory Findings===<br />
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.<br />
<br />
===Electrocardiogram===<br />
There are no ECG findings associated with adult T-cell leukemia. However, [[electrocardiogram]] might be helpful in detecting complications of adult T-cell leukemia such as [[Cardiac arrhythmia|cardiac arrhythmias]] due to [[hypercalcemia]]. To view the electrocardiogram findings in [[hypercalcemia]], click [[Hypercalcemia electrocardiogram|here]].<br />
<br />
===X-ray===<br />
There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia which include [[bone fractures]] and lytic lesions.<br />
<br />
=== Echocardiography and Ultrasound ===<br />
There are no [[echocardiography]] findings associated with adult T-cell leukemia. Findings on an [[ultrasound]] suggestive of adult T-cell leukemia include [[hepatomegaly]], [[splenomegaly]] and [[lymphadenopathy]].<br />
<br />
===CT Scan===<br />
[[Thoracic]] [[CT scan]] may be helpful in the diagnosis of adult T-cell leukemia. Findings on [[CT scan]] suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations. Findings on abdominal [[CT scan]] suggestive of adult T-cell leukemia cells include [[hepatomegaly]] and [[splenomegaly]].<br />
<br />
===MRI===<br />
There are no MRI findings associated with adult T-cell leukemia.<br />
<br />
=== Other Imaging Findings ===<br />
There are no other imaging findings associated with adult T-cell leukemia.<br />
<br />
===Other Diagnostic Studies===<br />
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization]]'''.'''<br />
<br />
==Treatment==<br />
===Medical Therapy===<br />
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell transplant]], or a combination of [[zidovudine]] and [[interferon]] therapy. While adult T-cell lymphoma patients are usually managed by either [[chemotherapy]], supportive care, or allogeneic [[stem cell]] transplant.<br />
<br />
===Interventions===<br />
There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.<br />
<br />
===Surgery===<br />
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.<br />
<br />
=== Primary Prevention ===<br />
Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of [[Human T-lymphotropic virus|HTLV]] virus. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.<br />
<br />
=== Secondary Prevention ===<br />
There are no established measures for the secondary prevention of Adult T-cell leukemia.<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.Jhttps://www.wikidoc.org/index.php?title=Adult_T-cell_leukemia_overview&diff=1502853Adult T-cell leukemia overview2018-11-12T20:07:21Z<p>Aida.J: /* Overview */</p>
<hr />
<div>__NOTOC__<br />
{{Adult T-cell leukemia}}<br />
{{CMG}} {{AE}} {{HL}}<br />
==Overview==<br />
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant<ref name="pmid30407885">{{cite journal |vauthors=Guru Murthy GS, Pondaiah SK, Abedin S, Atallah E |title=Incidence and survival of T-cell acute lymphoblastic leukemia in the United States |journal=Leuk. Lymphoma |volume= |issue= |pages=1–8 |date=November 2018 |pmid=30407885 |doi=10.1080/10428194.2018.1522442 |url=}}</ref><ref name="pmid28554708">{{cite journal |vauthors=Fernández MS, Rojas FD, Cattana ME, Mussin JE, de Los Ángeles Sosa M, Benzoni CD, Giusiano GE |title=Protothecosis in a patient with T cell lymphocytic leukemia |journal=Rev. Argent. Microbiol. |volume=49 |issue=3 |pages=224–226 |date=2017 |pmid=28554708 |doi=10.1016/j.ram.2017.02.007 |url=}}</ref>.<br />
<br />
==Historical Perspective==<br />
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981. <ref name="pmid27979862">{{cite journal |vauthors= |title=Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood. 1977;50(3):481-492 |journal=Blood |volume=128 |issue=24 |pages=2745 |date=December 2016 |pmid=27979862 |doi=10.1182/blood-2016-10-746677 |url=}}</ref><br />
<br />
==Classification==<br />
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<br />
<br />
==Pathophysiology==<br />
Adult T‐cell leukemia arises from post‐thymic [[Lymphocyte|lymphocytes]], which are normally involved in the process of [[Immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[Genetic mutation|genetic mutations]] induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[Nodule|nodules]], [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].<br />
<br />
==Causes==<br />
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[Genetic mutation|genetic mutations]] involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].<br />
<br />
==Differentiating Adult T-cell leukemia from other Diseases==<br />
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[Lymph node|lymph nodes]], such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<br />
<br />
==Epidemiology and Demographics==<br />
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.<br />
<br />
==Risk Factors==<br />
Common [[risk factors]] in the development of adult T-cell leukemia among [[HTLV]] carriers are [[vertical transmission]] of HTLV [[infection]] during [[Childbirth|labor]], male sex, and specific [[Human leukocyte antigen|human leukocyte antigens]] such as [[HLA-A]] 26, [[HLA-B]] 4002, and [[HLA-B]] 4801. <br />
<br />
==Screening==<br />
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for adult T-cell leukemia.<br />
<br />
==Natural History, Complications and Prognosis==<br />
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[Cardiac arrhythmia|cardiac arrhythmias]], [[Opportunistic infection|opportunistic infections]] , and [[Bone fracture|bone fractures]]. The [[prognosis]] varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor [[prognosis]], whereas chronic and smouldering subtypes have a good [[prognosis]].<br />
<br />
==Diagnosis==<br />
===Diagnostic Study of Choice===<br />
There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic [[histopathology]] and [[Human T-lymphotropic virus|HTLV]]-1 infection.<br />
<br />
===History and Symptoms===<br />
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and recurrent [[infection]]s.<br />
<br />
===Physical Examination===<br />
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].<br />
<br />
===Laboratory Findings===<br />
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.<br />
<br />
===Electrocardiogram===<br />
There are no ECG findings associated with adult T-cell leukemia. However, [[electrocardiogram]] might be helpful in detecting complications of adult T-cell leukemia such as [[Cardiac arrhythmia|cardiac arrhythmias]] due to [[hypercalcemia]]. To view the electrocardiogram findings in [[hypercalcemia]], click [[Hypercalcemia electrocardiogram|here]].<br />
<br />
===X-ray===<br />
There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia which include [[bone fractures]] and lytic lesions.<br />
<br />
=== Echocardiography and Ultrasound ===<br />
There are no [[echocardiography]] findings associated with adult T-cell leukemia. Findings on an [[ultrasound]] suggestive of adult T-cell leukemia include [[hepatomegaly]], [[splenomegaly]] and [[lymphadenopathy]].<br />
<br />
===CT Scan===<br />
[[Thoracic]] [[CT scan]] may be helpful in the diagnosis of adult T-cell leukemia. Findings on [[CT scan]] suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations. Findings on abdominal [[CT scan]] suggestive of adult T-cell leukemia cells include [[hepatomegaly]] and [[splenomegaly]].<br />
<br />
===MRI===<br />
There are no MRI findings associated with adult T-cell leukemia.<br />
<br />
=== Other Imaging Findings ===<br />
There are no other imaging findings associated with adult T-cell leukemia.<br />
<br />
===Other Diagnostic Studies===<br />
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization]]'''.'''<br />
<br />
==Treatment==<br />
===Medical Therapy===<br />
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell transplant]], or a combination of [[zidovudine]] and [[interferon]] therapy. While adult T-cell lymphoma patients are usually managed by either [[chemotherapy]], supportive care, or allogeneic [[stem cell]] transplant.<br />
<br />
===Interventions===<br />
There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.<br />
<br />
===Surgery===<br />
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.<br />
<br />
=== Primary Prevention ===<br />
Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of [[Human T-lymphotropic virus|HTLV]] virus. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.<br />
<br />
=== Secondary Prevention ===<br />
There are no established measures for the secondary prevention of Adult T-cell leukemia.<br />
<br />
==References==<br />
{{Reflist|2}}<br />
[[Category:Medicine]]<br />
[[Category:Hematology]]<br />
[[Category:Immunology]]<br />
[[Category:Infectious disease]]<br />
[[Category:Oncology]]<br />
[[Category:Up-To-Date]]<br />
[[Category:Primary care]]</div>Aida.J