Synonyms
Agnogenic myeloid metaplasia (AMM); Chronic idiopathic myelofibrosis (CIMF); Myelofibrosis with myeloid metaplasia
Definition
Primary myelofibrosis (PMF) is a clonal myeloproliferative neoplasm (MPN) characterized by a stepwise evolution from an initial prefibrotic/early phase (prePMF) 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 (Table 1). The initial stage of disease often is presenting with a marked thrombocytosis which clinically might mimic ET, but without sign of myeloid metaplasia. Fibrotic stages of PMF are clinically characterized by splenomegaly and leukoerythroblastosis in the blood with occurrence of teardrop-shaped red cells. One of the hallmarks of PMF is a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that in fully developed fibrotic disease stages is associated with hepatosplenomegaly,...
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References and Further Reading
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Tefferi, A., Guglielmelli, P., Larson, D. R., Finke, C., Wassie, E. A., Pieri, L., et al. (2014). Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood, 124(16), 2507–2513.
Thiele, J., Kvasnicka, H. M., Mullauer, L., Buxhofer-Ausch, V., Gisslinger, B., & Gisslinger, H. (2011). Essential thrombocythemia versus early primary myelofibrosis: A multicenter study to validate the WHO classification. Blood, 117(21), 5710–5718.
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Kvasnicka, H.M. (2020). Primary Myelofibrosis (PMF). In: Molina, T.J. (eds) Hematopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-95309-0_3853
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DOI: https://doi.org/10.1007/978-3-319-95309-0_3853
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