Myeloproliferative neoplasm: Difference between revisions

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{{DiseaseDisorder infobox |
{{Myeloproliferative neoplasm}}
  Name          = {{PAGENAME}} |
{{CMG}}{{AE}} {{MJK}} {{shyam}}
  Image          = |
  Caption        = |
  ICD10          = {{ICD10|D|47|1|d|37}} |
  ICD9          = {{ICD9|205.1}}, {{ICD9|238.4}}, {{ICD9|289.89}}, {{ICD9|289.9}} |
  ICDO          = 9950/0-9964/3  |
  OMIM          = |
  DiseasesDB    = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D009196 |
}}
{{SI}}
{{CMG}}
==Overview==


The '''myeloproliferative diseases''' ("MPD"s) are a group of diseases of the [[bone marrow]] in which excess cells are produced. They are related to, and may evolve into, [[myelodysplastic syndrome]] and [[acute myeloid leukemia]], although the myeloproliferative diseases on the whole have a much better prognosis than these conditions.
{{SK}} MPN; Myeloproliferative disease; Myeloproliferative disorder; Myeloproliferation
==Historical perspective==
The concept of myeloproliferative disease was first proposed in 1951 by the eminent hematologist [[William Dameshek]].<ref>{{cite journal | author = Dameshek W | title = Some speculations on the myeloproliferative syndromes. | journal = Blood | volume = 6 | issue = 4 | pages = 372-5 | year = 1951 | id = PMID 14820991}}</ref>


==Classification==
==[[Myeloproliferative neoplasm overview|Overview]]==
Although not a [[cancer|malignant neoplasm]], MPDs are classified within the [[Hematological malignancy|hematological neoplasms]].


There are four main myeloproliferative diseases, which can be further categorized by the presence of the [[Philadelphia chromosome]]:
==[[Myeloproliferative neoplasm historical perspective|Historical Perspective]]==


{| class="wikitable"
==[[Myeloproliferative neoplasm classification|Classification]]==
|-
! Philadelphia Chromosome "positive"
! Philadelphia Chromosome "negative"
|-
|
* [[Chronic myelogenous leukemia]] (CML)
|  
* [[Polycythemia vera]] (PV)
* [[Essential thrombocytosis]] (ET)
* [[Myelofibrosis]] (MF)
|-
|}


==Pathophysiology==
==[[Myeloproliferative neoplasm pathophysiology|Pathophysiology]]==


In 2005, the discovery of the JAK2 V617F mutation provided some evidence to suggest a common pathogenesis for the Philadelphia Chromosome negative MPDs.<ref>{{cite journal | author = Baxter EJ, Scott LM, Campbell PJ, et al. | title = Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. | journal = Lancet | volume = 365 | pages = 1054-1061 | year = 2005 | id = PMID 15781101}}</ref><ref>{{cite journal | author = James C, Ugo V, Le Couedic JP, et al. | title = A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. | journal = Nature | volume = 434 | issue = 7037 | pages = 1144–1148 | year = 2005 | id = PMID 15793561}}</ref><ref>{{cite journal | author = Levine RL, Wadleigh M, Cools J, et al. | title = Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis | journal = Cancer Cell | volume = 7 | issue = 4 | pages = 387–397  | year = 2005 | id = PMID 15837627}}</ref><ref>{{cite journal | author = Kralovics R, Passamonti F, Buser AS, et al. | title = A gain-of-function mutation of JAK2 in myeloproliferative disorders | journal = N Engl J Med | volume = 352 | issue = 17 | pages = 1779–1790 | year = 2005 | id = PMID 15858187}}</ref><ref>{{cite journal | author = Campbell PJ, Scott LM, Buck G, et al. | title = Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study | journal = Lancet | volume = 366 | issue = 9501 | pages = 1945–1953 | year = 2005 | id = PMID 16325696}}</ref>
==[[Myeloproliferative neoplasm causes|Causes]]==


==Causes==
==[[Myeloproliferative neoplasm differential diagnosis|Differentiating myeloproliferative neoplasm from other Diseases]]==
All MPDs arise from precursors of the "[[myeloid]]" lineage in the bone marrow.


==Differentiating Myeloproliferative disease from other Diseases==
==[[Myeloproliferative neoplasm epidemiology and demographics|Epidemiology and Demographics]]==
The lymphoid lineage may produce similar diseases, the [[lymphoproliferative disorder]]s ([[acute lymphoblastic leukemia]], [[lymphoma]]s, [[chronic lymphocytic leukemia]] and [[multiple myeloma]]).


== Diagnosis==
==[[Myeloproliferative neoplasm risk factors|Risk Factors]]==
Depending on the nature of the myeloproliferative disorder, diagnostic tests may include red cell mass determination (for polycythaemia), bone marrow aspirate and trephine biopsy, arterial oxygen saturation and carboxyhaemoglobin level, neutrophil alkaline phosphatase level, vitamin B12 (or B12 binding capacity) and serum urate.<ref>{{cite book |author=Levene, Malcolm I.; Lewis, S. M.; Bain, Barbara J.; Imelda Bates |title=Dacie & Lewis Practical Haematology |publisher=W B Saunders |location=London |year= |pages= |isbn=0-443-06377-X |pages=586}}</ref>


==References==
==[[Myeloproliferative neoplasm screening|Screening]]==  
{{Reflist|2}}


== External links ==
==[[Myeloproliferative neoplasm natural history, complications, and prognosis|Natural History, Complications, and Prognosis]]==
* [http://www.mpdinfo.org/ Myeloproliferative Disorders Website of The CMPD Education Foundation]
*[http://www.mpdfoundation.org MPD Foundation and Research Alliance - Register for a free newsletter]


{{Hematology}}
==Diagnosis==
{{Hematological malignancy histology}}
[[Myeloproliferative neoplasm diagnostic study of choice|Diagnostic study of choice]] | [[Myeloproliferative neoplasm history and symptoms|History and Symptoms]] | [[Myeloproliferative neoplasm physical examination|Physical Examination]] | [[Myeloproliferative neoplasm laboratory findings|Laboratory Findings]] | [[Myeloproliferative neoplasm chest x ray|Chest X Ray]] |  [[Myeloproliferative neoplasm CT|CT]] | [[Myeloproliferative neoplasm MRI|MRI]] | [[Myeloproliferative neoplasm ultrasound|Ultrasound]] | [[Myeloproliferative neoplasm other imaging findings|Other Imaging Findings]] | [[Myeloproliferative neoplasm other diagnostic studies|Other Diagnostic Studies]]


[[Category:Hematology]]
==Treatment==
[[Myeloproliferative neoplasm medical therapy|Medical Therapy]] | [[Myeloproliferative neoplasm surgery|Surgery]] | [[Myeloproliferative neoplasm primary prevention|Primary Prevention]] | [[Myeloproliferative neoplasm secondary prevention|Secondary Prevention]] | [[Myeloproliferative neoplasm cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Myeloproliferative neoplasm future or investigational therapies|Future or Investigational Therapies]]


[[ar:مرض التكاثر النقوي]]
==Case Studies==
[[de:Myeloproliferative Erkrankung]]
[[Myeloproliferative neoplasm case study one|Case #1]]
[[es:Síndrome mieloproliferativo crónico]]
[[fr:Syndrome myéloprolifératif]]
[[pl:Zespoły mieloproliferacyjne]]


 
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Latest revision as of 22:50, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2] Shyam Patel [3]

Synonyms and keywords: MPN; Myeloproliferative disease; Myeloproliferative disorder; Myeloproliferation

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating myeloproliferative neoplasm from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

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Case #1