Progress of molecular pathogenesis and treatment in polycythemia vera

VernacularTitle:真性红细胞增多症分子生物学发病机制及治疗研究进展
Author: Yaru LI ; Wei CHEN
Keywords: Polycythemia vera; Molecular biology; JAK2 inhibitor
From: Journal of Leukemia & Lymphoma 2017;26(7):440-444
CountryChina
Language:Chinese
Abstract: Polycythemia vera (PV) is a chronic clonal myeloproliferative disorder of hematopoietic stem cell characterized by a pronounced symptom burden, including fatigue, pruritus, and symptomatic splenomegaly, along with an increased risk of thrombosis and the potential for evolution to myelofibrosis (MF) and secondary acute myeloid leukemia. Research on pathogenesis, diagnosis and treatment of PV has made a great progress since the discovery of JAK2 V617F mutation in 2005. The change of epigenetics plays an important role in the progression of PV, and the drugs for apparent genetic alteration may improve the life quality of PV patients.