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]
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" |
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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
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- ^ 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.
acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH
aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
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Hematological malignancy histology (ICD-O 9590-9989) |
| Lymphomas (9590-9759) |
Hodgkin's lymphoma vs. Non-Hodgkin lymphoma - Diffuse lymphoma vs. Follicular lymphoma
B-cell lymphoma (Small cell, Primary effusion, Diffuse large, ,Burkitt's, Splenic marginal zone, MALT)
T-cell lymphoma (Cutaneous , Mycosis fungoides/Sézary's disease, Angioimmunoblastic, Anaplastic large cell, Hepatosplenic)
plasma cell (Plasmacytoma, Multiple myeloma)
mast cell tumor (Mast-cell sarcoma, Malignant mastocytosis, Malignant histiocytosis, Langerhans cell histiocytosis) |
| Immunoproliferative disorders (9760-9799) |
Waldenström macroglobulinemia - Lymphomatoid granulomatosis |
| Lymphoid leukemias (9800-9839) |
ALL - CLL - T-cell leukemia (Adult, Large granular lymphocyte, Prolymphocytic, Acute lymphoblastic) - B-cell leukemia (Prolymphocytic) |
| Myeloid leukemias (9840-9939, 9963) |
AML (M2, APL/M3, AMoL/M5, Erythroleukemia/M6) - CML (CMoL, CNL, Philadelphia chromosome) - Granulocytic sarcoma |
| Other leukemias (9940-9949) |
Hairy cell leukemia - Aggressive NK-cell leukemia |
| Myeloproliferative disease (9950-9961) |
Polycythemia vera - Essential thrombocytosis - Myelofibrosis |
| Other (9964-9989) |
Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome |
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