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Myeloproliferative disease



Myeloproliferative disease
Classification & external resources
ICD-10 D47.1
ICD-9 205.1, 238.4, 289.89, 289.9
ICD-O: 9950/0-9964/3
MeSH D009196

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]

Additional recommended knowledge

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"

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. ^ Dameshek W (1951). "Some speculations on the myeloproliferative syndromes.". Blood 6 (4): 372-5. PMID 14820991.
  2. ^ 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.
  3. ^ 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.
  4. ^ 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.
  5. ^ 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.
  6. ^ 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.

acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH

aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
Coagulation/plateletscoagulopathy: DIC • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease

Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP

primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Antiphospholipid syndrome - Factor V Leiden

other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome
HistiocytosisWHO-I Langerhans cell histiocytosis - non-Langerhans-cell histiocytosis/WHO-II (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) - malignant histiocytic disorders/WHO-III (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
OtherAsplenia/hyposplenism - Methemoglobinemia
  This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Myeloproliferative_disease". A list of authors is available in Wikipedia.
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