Myeloproliferative neoplasm
Template:DiseaseDisorder infobox
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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.
Historical perspective
The concept of myeloproliferative disease was first proposed in 1951 by the eminent hematologist William Dameshek.[1]
Classification
Although not a malignant neoplasm, MPDs are classified within the hematological neoplasms.
There are four main myeloproliferative diseases, which can be further categorized by the presence of the Philadelphia chromosome:
| Philadelphia Chromosome "positive" | Philadelphia Chromosome "negative" |
|---|---|
|
Pathophysiology
In 2005, the discovery of the JAK2 V617F mutation provided some evidence to suggest a common pathogenesis for the Philadelphia Chromosome negative MPDs.[2][3][4][5][6]
Causes
All MPDs arise from precursors of the "myeloid" lineage in the bone marrow.
Differentiating Myeloproliferative disease from other Diseases
The lymphoid lineage may produce similar diseases, the lymphoproliferative disorders (acute lymphoblastic leukemia, lymphomas, chronic lymphocytic leukemia and multiple myeloma).
Diagnosis
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.<refLevene, Malcolm I.; Lewis, S. M.; Bain, Barbara J.; Imelda Bates. Dacie & Lewis Practical Haematology. London: W B Saunders. p. 586. ISBN 0-443-06377-X.</ref>
References
- ↑ Dameshek W (1951). "Some speculations on the myeloproliferative syndromes". Blood. 6 (4): 372–5. PMID 14820991.
- ↑ Baxter EJ, Scott LM, Campbell PJ; et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365: 1054–1061. PMID 15781101.
- ↑ James C, Ugo V, Le Couedic JP; et al. (2005). "A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera". Nature. 434 (7037): 1144–1148. PMID 15793561.
- ↑ Levine RL, Wadleigh M, Cools J; et al. (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell. 7 (4): 387–397. PMID 15837627.
- ↑ Kralovics R, Passamonti F, Buser AS; et al. (2005). "A gain-of-function mutation of JAK2 in myeloproliferative disorders". N Engl J Med. 352 (17): 1779–1790. PMID 15858187.
- ↑ Campbell PJ, Scott LM, Buck G; et al. (2005). "Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study". Lancet. 366 (9501): 1945–1953. PMID 16325696.
External links
- Myeloproliferative Disorders Website of The CMPD Education Foundation
- Myeloproliferative Disorders in practice
- Myeloproliferative Disease Support List
- Myeloproliferative+Disorders at the US National Library of Medicine Medical Subject Headings (MeSH)
- MPD Foundation and Research Alliance - Register for a free newsletter
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