9961/3 Primary myelofibrosis


Myelosclerosis with myeloid metaplasia
Megakaryocytic myelosclerosis
Myelofibrosis with myeloid metaplasia
Agnogenic myeloid metaplasia
Chronic idiopathic myelofibrosis
Myelofibrosis as a result of myeloproliferative disease


Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: D47.4

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Allred DC, Bryant J, Paik S, Fisher E, Julian T, Margolese R, Smith R, Mamounas T, Osbourne CK, Fisher B, Wolmark N (2002) ()
Estrogen receptor expression as a predictive marker of the effectiveness of tamoxifen in the treatment of DCIS
Findings from the NSABP Protocol B-24. Breast Cancer Res Treat 76 (Suppl 1): A30.

Primary myelofibrosis (PMF) 2
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Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A, (2007)
Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT).
Leuk Res 31: 737-40

is a clonal myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow (BM) that in fully developed disease is associated with reactive deposition of fibrous connective tissue and with extra?medullary haema?topoiesis (EMH). There is a stepwise evolution from an initial prefibrotic phase 3
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Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA, Barosi G, Verstovsek S, Birgegard G, Mesa R, Reilly JT, Gisslinger H, Vannucchi AM, Cervantes F, Finazzi G, Hoffman R, Gilliland DG, Bloomfield CD, Vardiman JW (2007)
Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel.
Blood 110: 1092-7

characterized by a hypercellular BM with absent or minimal reticulin fibrosis to a fibrotic phase with marked reticulin or collagen fibrosis in the BM and often osteo?sclerosis. This fibrotic stage of PMF is characterized by leukoerythroblastosis in the blood with teardrop-shaped red cells, and by hepatomegaly and splenomegaly. The diagnostic criteria are outlined in (Table 2.04).