Abstract
The BCR-ABL-negative myeloproliferative neoplasms (MPN) are a group of clonal hematopoietic stem cell disorders characterized by the increased proliferation of granulocytic, megakaryocytic, or erythroid cells; that include polycythemia vera (PV), essential thrombocytopenia (ET) and myelofibrosis (MF), which consist of both primary myelofibrosis (PMF) and myelofibrotic transformation from PV or ET to post-PV/ET myelofibrosis (post ET/PV MF).1-3 Patients with PV present with increased erythroid counts, low serum erythropoietin levels, sometimes splenomegaly, and a higher risk of thrombotic and microvascular events. While myelofibrosis typically presents with increased bone marrow fibrosis, cytopenias, progressive hepatosplenomegaly, extramedullary hematopoiesis, constitutional symptoms, such as night sweats, fever, and weight loss; and an increased risk for leukemic transformation.
Published Version
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