logo logo
Search: Organ site Search: Tumor / Syndrome
or
WHO Classification of Tumours
Primary myelofibrosis
Tumours of haematopoietic and lymphoid tissues


Definition

Primary myelofibrosis (PMF)
Click to access Pubmed
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 extra­medullary haema­topoiesis (EMH). There is a stepwise evolution from an initial prefibrotic phase
Click to access Pubmed
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 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).

> Related Topics
Essential thrombocythemia
Polycythemia vera
Acute panmyelosis with myelofibrosis
Introduction: Myeloid Neoplasms