Janus激酶抑制剂治疗Ph -骨髓增殖性肿瘤相关骨髓纤维化患者的研究现状

Research status of Janus kinase inhibitors in treatment of patients with Ph-myeloproliferative neoplasms associated myelofibrosis

Ph-骨髓增殖性肿瘤(MPN)相关骨髓纤维化(MF)包括原发性MF(PMF),真性红细胞增多症后MF(post-PV MF)和原发性血小板增多症后MF(post-ET MF)。自首个MF疾病始动主要分子途径Janus激酶(JAK)-信号转导和转录激活因子(STAT)信号通路抑制剂芦可替尼问世以来,其他JAK抑制剂(JAKi)新药陆续获批用于治疗Ph-MPN相关MF患者,这使MF患者的治疗推荐和路径不断更新。笔者拟就JAKi在Ph-MPN相关MF患者的一、二线治疗中的研究现状进行阐述,旨在为JAKi的合理临床使用提供参考。

Ph-myeloproliferative neoplasms(MPN)associated myelofibrosis(MF)includes primary MF(PMF),post-positive erythrocytosis MF(post-PV MF),and post-primary thrombocytosis MF(post-ET MF).Since the advent of ruxolitinib,the first MF disease-initiating major molecular pathway Janus kinase(JAK)/signal transducer and activator of transcription(STAT)signaling pathway inhibitor,other new JAK inhibitor(JAKi)have been approved for the treatment of Ph-MPN associated MF patients.And Ph-MPN associated MF treatment recommendations and pathways were constantly being updated.This article intends to elaborate on current research status of JAKi application in the first-and second-line treatment for patients with Ph-MPN associated MF,aiming to provide a reference for the rational clinical use of JAKi.