摘要骨髓纤维化(MF)是一种骨髓增殖性肿瘤,目前针对该病的有效治疗方法很少.随着JAK2V617F基因突变的发现及非受体酪氨酸激酶Janus激酶(JAK)抑制剂的问世,为MF患者提供新的治疗选择.芦可替尼(ruxolitinib)是首个被美国食品与药品监督管理局(FDA)批准上市的选择性JAK1/2抑制剂,适用于国际预后积分系统(IPSS)中危-2组及高危组MF患者,可有效改善患者体质性症状,缩小脾脏体积,延长患者生存期.但是,该药物在临床应用中,也存在药物相关不良反应,如血细胞减少、感染、继发第2肿瘤等,以及对部分患者治疗失败等问题.笔者拟主要从芦可替尼治疗MF患者的药物相关不良反应,芦可替尼对MF治疗失败的定义、原因、高危因素及预后,以及治疗失败后的挽救性治疗的最新研究进展进行综述.

abstractsMyelofibrosis (MF)is a type of myeloproliferative neoplasms which is difficult to be treated.With the discovery of V617F mutation in Janus kinase (JAK)2,JAK inhibitor provides a new treatment strategy for patients with myelofibrosis.Ruxolitinib is the first JAK1/2 inhibitor which approved by the Food and Drug Administration (FDA)for the treatment of patients with international prognostic scoring system (IPSS )intermediate-2 or high risk myelofibrosis.In clinical experiments, ruxolitinib demonstrates significant improvements in splenomegaly and disease-related constitutional symptoms,and prolongs patients'overall survival.However,the side effects of the drug (hematocytopenia,infections and secondary neoplasms)and treatment failure of some patients has been observed in treatments.The adverse reaction and solutions of ruxlitinib;the definition,reason,high risk factors and prognosis of treatment failure;the salvage therapy after treatment failure will be discussed in this review.