Tumours of haematopoietic and lymphoid tissues
Primary myelofibrosis (PMF)
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)
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 extramedullary haematopoiesis (EMH). There is a stepwise evolution from an initial prefibrotic phase
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
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)
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 osteosclerosis. 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).
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
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Acute panmyelosis with myelofibrosis
Introduction: Myeloid Neoplasms