原发性骨髓纤维化(primary myelofibrosis,PMF)是一种慢性克隆性髓系疾病,特点是贫血、脾肿大、骨髓纤维化、骨硬化,可伴随发热、盗汗、乏力、消瘦等全身症状~([1])。其预后差,死亡的主要原因为感染、出血以及转化为急性白血病,中位生存期约为5年。目前PMF的治疗缺乏特异性高的药物,治疗方法主要是改善贫血、免疫调节剂如沙利度胺或雷利度胺或干扰素等、羟基脲、JAK2V617F Primary myelofibrosis (PMF) is a chronic myeloid disease characterized by anemia, splenomegaly, myelofibrosis and osteosclerosis, which may be accompanied by systemic symptoms of fever, night sweats, fatigue, weight loss, ([1]). The prognosis is poor, the main cause of death is infection, bleeding and conversion to acute leukemia, the median survival of about 5 years. The current lack of treatment of PMF specific high drug, the treatment is mainly to improve anemia, immune regulators such as thalidomide or lenalidomide or interferon, hydroxyurea, JAK2V617F