摘要原发性骨髓纤维化(PMF)属于慢性骨髓增殖性肿瘤的一种,JAK2V617F突变及其他分子学异常奠定了大多数费城染色体阴性的骨髓增殖性肿瘤的发病基础.从2005年第一次描述JAK2V617F突变到近期发现钙网蛋白(CALR)突变,再到JAK激酶抑制剂的临床应用,展示了对骨髓纤维化发病机制研究的深入和治疗手段的提高.现就2014年欧洲血液学会(EHA)年会上关于PMF的诊断、危险度分层、JAK激酶抑制剂治疗的益处和潜在毒副作用以及联合治疗的研究现状进行综述.

abstractsPrimary myelofibrosis is a kind of chronic myeloproliferative neoplasms.The discovery of the JAK2V617F mutation as well as other molecular abnormlities underly the pathogenesis of Philadelphia negative myeloproliferative neoplasms (MPN).The initial description of JAK2V617F mutation in 2005,the reporting of calreticulin (CALR) mutations last year and the clinical application of JAK inhibitors,showed the gradually deepening understanding with regard to the pathogenesis and the development of therapeutic strategies for primary myelofibrosis (PMF).This article reviewed the progress in diagnosis,risk stratification of of PMF,the benefits and potential side effect of JAK inhibitors,which reported on the 2014 European Hematology Association annual congress.