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==[[Malignant histiocytosis overview|Overview]]==
 
==[[Malignant histiocytosis pathophysiology |Pathophysiology]]==
==Overview==
==[[Malignant histiocytosis causes|Causes]]==
'''Malignant histiocytosis''' is a hereditary disease found in the Bernese Mountain Dog characterized by [[histiocyte|histiocytic]] infiltration of the lungs and [[lymph node]]s.  The [[liver]], [[spleen]], and [[central nervous system]] can also be affected.  Histiocytes are a component of the [[immune system]] that proliferate abnormally in this disease.  In addition to its importance in veterinary medicine, the condition is also important in human pathology.<ref>http://dermnetnz.org/dermal-infiltrative/malignant-histiocytoses.html</ref>
==[[Malignant histiocytosis differential diagnosis|Differentiating Malignant histiocytosis from other Diseases]]==
 
==[[Malignant histiocytosis epidemiology and demographics|Epidemiology and Demographics]]==
==Histiocytic disorders==
==[[Malignant histiocytosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
A histiocyte is a differentiated tissue cell that has its origin in the [[bone marrow]].  The source for histiocytes is the [[monocyte]]/[[macrophage]] line.  Monocytes (found in the blood) and macrophages (found in tissue) are responsible for [[phagocytosis]] (ingestion) of foreign material in the body.  [[Langerhans cell]]s are [[dendritic cell]]s found in the skin and function by internalizing [[antigen]]s (foreign particles) and presenting them to [[T cell]]s.  They arise from monocytes.<ref>{{cite journal |author=Ginhoux F, Tacke F, Angeli V, Bogunovic M, Loubeau M, Dai XM, Stanley ER, Randolph GJ, Merad M |title=Langerhans cells arise from monocytes in vivo |journal=Nat. Immunol. |volume=7 |issue=3 |pages=265-73 |year=2006 |pmid=16444257}}</ref>  Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells.  They include the following:
==Diagnosis==
*Reactive diseases of Langerhans cells
[[Malignant histiocytosis history and symptoms|History and Symptoms]] | [[Malignant histiocytosis physical examination|Physical Examination]] | [[Malignant histiocytosis laboratory findings|Laboratory Findings]] | [[Malignant histiocytosis CT|CT]] | [[Malignant histiocytosis MRI|MRI]] | [[Malignant histiocytosis other imaging findings|Other Imaging Findings]] | [[Malignant histiocytosis other diagnostic studies|Other Diagnostic Studies]]
**[[Histiocytoma (dog)|Histiocytoma]]s
**Cutaneous [[histiocytosis]]
**Systemic histiocytosis
*Reactive diseases of macrophages
**Hemophagocytic syndrome - a condition where macrophages phagocytose [[myeloid]] and [[erythroid]] precursors (similar to [[HLH (Hemophagocytic lymphohistiocytosis)|hemophagocytic lymphohistiocytosis]] in humans)
*Malignant diseases of Langerhans dells
**Malignant histiocytosis
**Diffuse histiocytic sarcoma
**Localized histiocytic sarcoma
*Malignant diseases of macrophages
**Histiocytic lymphoma
 
==Breed disposition==
Up to 25 percent of Bernese Mountain Dogs may develop malignant histiocytosis in their lifetime.<ref>{{cite web|author=Parker, A.G., Rutteman, G.R., Cadieu, E., et al.|year=2006|title=Malignant Histiocytosis in the Bernese Mountain Dog: Design and Execution of a Case-Control Study|work=Genes, Dogs and Cancer: Fourth Annual Canine Cancer Conference| url=http://www.ivis.org/proceedings/keystone/2006/sessionB/parker/chapter.asp?la=1|accessdate=2007-01-28}}</ref>  Other breeds with a possible genetic tendency toward malignant histiocytosis include Rottweilers, Flat-Coated Retrievers, and Golden Retrievers<ref name=Ettinger_1995>{{cite book|author=Ettinger, Stephen J.;Feldman, Edward C.|title=Textbook of Veterinary Internal Medicine|edition=4th ed.|publisher=W.B. Saunders Company|year=1995|id=ISBN 0-7216-6795-3}}</ref>. 
 
==Signs and symptoms==
The disease in the lungs is characterized by enlargement of the tracheobronchial lymph nodes and infiltration of the lungs, sometimes leading to lung lobe consolidation and [[pleural effusion]].<ref>{{cite web|author=Kessler, Martin|year=2006|title=Radiographic diagnosis of primary and metastatic lung tumors|work=Proceedings of the North American Veterinary Conference| url=http://www.ivis.org/proceedings/navc/2006/SAE/275.pdf?LA=1|accessdate=2007-01-28}}</ref>  Signs and symptoms include cough, loss of appetite, weight loss, anemia, and difficulty breathing.<ref>{{cite web | last = Affolter | first = Verena K. | title = Histiocytic Proliferative Diseases in Dogs and Cats | work = Proceedings of the 29th World Congress of the World Small Animal Veterinary Association | date = 2004 | url = http://www.vin.com/proceedings/Proceedings.plx?CID=WSAVA2004&PID=8600&O=Generic | accessdate = 2007-01-28 }}</ref>  [[Seizure]]s and rear limb weakness can be seen.  Invasion of the bone marrow can cause [[pancytopenia]].<ref>{{cite journal | last = Kearns | first = Shawn Ann | coauthors = Ewing, Patty | title = Causes of canine and feline pancytopenia | journal = Compendium on Continuing Education for the Practicing Veterinarian | volume = 28 | issue = 2 | pages = 122-133 | publisher = Veterinary Learning Systems | date = Feb. 2006 }}</ref>  Diagnosis requires a [[biopsy]]


==Treatment==
==Treatment==
Treatment with [[chemotherapy]] has been used with some success, particularly using [[lomustine]], [[prednisone]], [[doxorubicin]], and [[cyclophosphamide]].<ref name=DVM>{{cite journal | last = Cronin | first = Kim | title = Deciphering the histiocytic code | journal = DVM | pages = 1S-8S | publisher = Advanstar Communications | date = Dec. 2006 }}</ref>  Because of the rapid progression of this aggressive disease, the [[prognosis]] is very poor.<ref>{{cite journal |author=Affolter V, Moore P |title=Localized and disseminated histiocytic sarcoma of dendritic cell origin in dogs |journal=Vet Pathol |volume=39 |issue=1 |pages=74-83 |year=2002 |id=PMID 12102221}}</ref>
[[Malignant histiocytosis medical therapy|Medical Therapy]] | [[Malignant histiocytosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Malignant histiocytosis future or investigational therapies|Future or Investigational Therapies]]
==Case Studies==
:[[Malignant histiocytosis case study one|Case #1]]


==Other histiocytic diseases in Bernese Mountain Dogs==
==Related chapters==
A similar disease is '''diffuse histiocytic sarcoma''', a term used to designate a localized histiocytic sarcoma that has spread throughout the body.<ref name=DVM/>
 
Another disease of histiocytic origin that affects Bernese Mountain Dogs is '''systemic histiocytosis'''.  This condition generally begins as lesions on the [[eyelid]]s, nasal [[mucous membrane|mucosa]], and skin, especially the [[scrotum]].  It progresses to a more generalized disease affecting the lymph nodes, bone marrow and spleen.<ref>{{cite web | title = Tumors with Histiocytic Differentiation | work = The Merck Veterinary Manual | date = 2006 | url = http://www.merckvetmanual.com/mvm/index.jsp?cfile=htm/bc/72232.htm | accessdate = 2007-01-28 }}</ref>  Other signs and symptoms include weight loss and loss of appetite.<ref name=DVM/>  It also has a very poor prognosis.
 
==See also==
*[[Histiocytosis]]
*[[Histiocytosis]]
==References==
{{Reflist|2}}


{{Hematology}}
{{Hematology}}
{{Hematological malignancy histology}}
{{Hematological malignancy histology}}


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Latest revision as of 18:02, 21 September 2012

Malignant histiocytosis
ICD-10 C96.1
ICD-9 202.3
ICD-O: 9750/3
MeSH D006647

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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