Abstract
This review details two major clonal stem cell disorders: essential thrombocythemia (ET) and myelofibrosis (MF). ET is distinguished by a sustained proliferation of megakaryocytes that results in peripheral blood thrombocytosis. Primary myelofibrosis (PMF) is associated with extramedullary hematopoiesis, splenomegaly, a leukoerythroblastic blood picture, and varying degrees of marrow fibrosis with marked megakaryocyte hyperplasia and atypia. The epidemiology, etiology/genetics, pathogenesis, diagnosis (including clinical manifestations and laboratory tests), differentials, management, and prognosis of each disorder are examined. Also included is the evaluation of treatment options for MF, including interferon alfa, JAK inhibitors, and allogeneic stem cell transplantation, the latter of which is still the only curative treatment for MF. Figures show treatment algorithms for ET and MF. Tables list the current criteria for the diagnosis of ET and PMF via the World Health Organization (WHO), the guidelines for diagnosis of post-ET MF via the International Working Group for Myelofibrosis Research and Treatment (IWG-MRT), prognostic factors in the International Prognostic Score for ET (IPSET) and IPSET-thrombosis, prognostic scoring systems for MF, and the clinical activity of JAK2 inhibitors. This review contains 2 highly rendered figures, 6 tables, and 60 references.
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