Discover

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. The concept of myeloproliferative disease was first proposed in 1951 by the eminent hematologist William Dameshek.^[1]

Contents

Classification

Causes

References

External links

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"
★ Chronic myelogenous leukemia (CML)	★ Polycythemia vera (PV) ★ Essential thrombocytosis (ET) ★ Myelofibrosis (MF)

Causes

All MPDs arise from precursors of the "myeloid" lineage in the bone marrow. The lymphoid lineage may produce similar diseases, the lymphoproliferative disorders (acute lymphoblastic leukemia, lymphomas, chronic lymphocytic leukemia and multiple myeloma).
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]}

References

1. Some speculations on the myeloproliferative syndromes., Dameshek W, , , Blood, 1951
2. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders., Baxter EJ, Scott LM, Campbell PJ, et al., , , Lancet, 2005
3. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera., James C, Ugo V, Le Couedic JP, et al., , , Nature, 2005
4. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis, Levine RL, Wadleigh M, Cools J, et al., , , Cancer Cell, 2005
5. A gain-of-function mutation of JAK2 in myeloproliferative disorders, Kralovics R, Passamonti F, Buser AS, et al., , , N Engl J Med, 2005
6. Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study, Campbell PJ, Scott LM, Buck G, et al., , , Lancet, 2005

External links

★ Myeloproliferative Disorders Website of The CMPD Education Foundation

★ Myeloproliferative Disorders in practice

★ Myeloproliferative Disease Support List

★

★ MPD Foundation and Research Alliance