Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management

American Journal of Hematology
Ayalew Tefferi

Abstract

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR or MPL mutations; additional disease features include bone marrow reticulin/collagen fibrosis, aberrant inflammatory cytokine expression, anemia, hepatosplenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression and shortened survival. Bone marrow morphology is the primary basis for diagnosis. Presence of JAK2, CALR or MPL mutation, expected in ̴ 90% of the patients, is supportive but not essential for diagnosis; these mutations are also prevalent in the closely related MPNs, namely polycythemia vera (PV) and essential thrombocythemia (ET). The 2016 World Health Organization classification system distinguishes "prefibrotic" from "overtly fibrotic" PMF; the former might mimic ET in its presentation. Furthermore, approximately 15% of patients with ET or PV might progress into a PMF-like phenotype (post-ET/PV MF), during their clinical course. SRSF2, ASXL1 and U2AF1-Q157 mutations predict inferior survival in PMF, independent of each other and other risk factors. RAS/CBL mutations predicted resistance to r...Continue Reading

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Citations

Mar 3, 2021·Clinical Lymphoma, Myeloma & Leukemia·Shelby MeckstrothNikolai Podoltsev
Apr 4, 2021·International Journal of Molecular Sciences·Theodoros KarantanosAmy E DeZern
May 31, 2021·Transplantation and Cellular Therapy·Jan Philipp BewersdorfMaximilian Stahl
Aug 8, 2021·International Journal of Molecular Sciences·Liberato GiardulloFrancesco Paolo Cantatore
Sep 10, 2021·American Journal of Hematology·Sarah E GreenMaria R Baer

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