原发性血小板增多症、真性红细胞增多症和骨髓纤维化:目前的处理和靶向治疗  

Essential thrombocythemia,polycythemia vera,and myelofibrosis:Current management and the prospect of targeted therapy

作　　者：王学文[1] 

机构地区：[1]南京军区南京总医院血液病科,江苏南京210002

出　　处：《现代肿瘤医学》2009年第10期2008-2011,共4页Journal of Modern Oncology

摘　　要：最近发现JAK2和/或MPL突变对真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF)的诊断和治疗产生着重大影响。例如,JAK2突变目前认为是诊断PV的必要条件,WHO分类系统最近修订的PV、ET和PMF诊断标准中包括JAK2和MPL(血小板生成素受体)突变作为克隆性标记。从治疗学的观点,JAK-STAT信号途径现已确定为研发骨髓增殖性新生物治疗新药的合理靶途径。本文简述ET、PV和PMF目前的处理,并复习抗JAK2小分子药物临床前和临床活性的有关资料。The recent discovery of JAK2 and/or MPL mutations in polycythemia vera （PV） , essential thrombocythemia （ET） ,and primary myelofibrosis （PMF） has had a major impact on how we diagnose and treat these disorders. For instance,the presence of a JAK2 mutation is now considered necessary for the diagnosis of PV and the World Health Organization classification system has recently revised its diagnostic criteria for PV, ET and PMF to include JAK2 and MPL mutations as elonal markers. From the standpoint of treatment,JAK- STAT is now identified as a legitimate target pathway for drug development in myeloproliferative neoplasms, we will first outline the views regarding current management in ET, PV and PMF and then discuss emerging data on preclinical and clinical activity of anti - JAK2 small molecule drugs.

关 键 词：真性红细胞增多症 原发性血小板增多症 原发性骨髓纤维化 JAK2 MPL 

分 类 号：R730.3[医药卫生—肿瘤]