骨髓增殖性肿瘤分子发病机制及治疗研究进展

Development of pathogenesis and therapeutics in myeloproliferative neoplasms

介绍了第56届美国血液学会（ASH）年会前后报道的一些骨髓增殖性肿瘤（MPN）相关新基因及目前国内外治疗MPN的现状.强调MPN治疗前需要进行风险评分,原发性血小板增多症（ET）的风险评分可采用ET国际预后积分（IPSET）算法,真性红细胞增多症（PV）的风险评分可采用Tefferi算法,原发性骨髓纤维化（PMF）的风险评分可采用动态国际预后评分系统加强版（DIPSS-plus）算法.总结了异基因造血干细胞移植、静脉放血治疗、血细胞去除治疗及药物治疗的进展,尤其关注抗凝药物阿司匹林、阿那格雷、针对JAK2基因的靶向药物芦可替尼以及国内治疗PMF的主要药物沙利度胺.

This article introduces the genes related to pathogenesis and prognosis of myeloproliferative neoplasms （MPN） and the current situation of MPN domestic and overseas,which have been reported in the 56th ASH annual meeting.It is emphasized on essential evaluation of the risk score using IPSET in ET,Tefferi in PV and DIPSS-plus in PMF separately.It has assessed advancements in allogeneic hematopoietic stem cell transplantation,phlebotomy,cytoreductive therapy and drug therapy.The article pays more attention to aspirin,anagrelide,JAK2 targeted agent ruxolitinib and thalidomide which is domestically used in the treatment of PMF.