摘要
Abstract
Essential thrombocythemia(ET) is often regarded as the most indolent of "BCR-ABL1-negative MPN',a disorder which characterized by stem cell-derived clonal myeloproliferation with mutually exclusive JAK2,CALR and MPL mutations.Accurate distinction between ET and pre-fibrotic/early primary myelofibrosis (pre PMF) is important in terms of both prognostication and treatment.In strictly WHO-defined ET,because the survival is near-normal,it would be inappropriate to suggest that any treatments modify the natural history of the disease.In contrast,the goal of current therapy in ET is primarily to prevent thrombohemorrhagic complications and secondarily to control the microcirculatory symptoms.In this regard,treatment is tailored to individual patients according to their risk for thrombosis or bleeding.Ruxolitinib is the first JAK inhibitor approved in the world with comfirmed clinical benefit to other MPNs,but the long-term efficacy of ruxolitinib treatment for ET is still lacking evidence.关键词
原发性血小板增多症/PMF,MF前或早期/国际预后指数/JAK抑制剂Key words
essential thrombocythemia/pre-fibrotic/early primary myelofibrosis/international prognostic score/JAK inhibitor分类
医药卫生
