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Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management
Author(s) -
Tefferi Ayalew
Publication year - 2018
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.25230
Subject(s) - myelofibrosis , international prognostic scoring system , myeloproliferative neoplasm , medicine , oncology , hepatosplenomegaly , bone marrow , constitutional symptoms , essential thrombocythemia , disease , polycythemia vera , myelodysplastic syndromes
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 stromal reaction including reticulin fibrosis, abnormal cytokine expression, anemia, hepatosplenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression, and shortened survival.