联合免疫抑制治疗难治重型再生障碍性贫血预后不良危险因素研究

Risk factors related to poor prognosis in refractory severe aplastic anemia cases after integrated immunosuppressive therapy

目的探讨对联合免疫抑制治疗(IST)难治的重型再生障碍性贫血(SAA)患者预后不良的危险因素。方法应用多因素分析方法回顾1996年12月至2006年10月中国医学科学院血液病医院154例SAA患者性别、年龄、病因、诊断至IST的时间、疾病严重程度(发病时中性粒细胞绝对值)、发病时感染、抗胸腺细胞球蛋白(ATG)剂型、粒细胞集落刺激因子(G-CSF)的应用以及阵发性睡眠性血红蛋白尿(PNH)克隆等因素与IST治疗后的难治性及生存情况的相关性。结果多因素分析显示,疾病的严重程度、感染是SAA患者对IST难治和预后不良的危险因素。G-CSF应用>6个月发生包括单体7在内的克隆性染色体转化即骨髓增生异常综合征(MDS)转化的危险性增高。结论疾病的严重程度及感染发生是SAA患者对IST难治和预后不良的主要危险因素。G-CSF应用时间大于6个月是MDS转化的危险因素。

Objective To investigate the risk factors related to poor prognosis in refractory severe aplastic anemia （SAA） patients after immunosuppressive therapy. Methods Multivariate analysis was used to analyze the correlation of post-IST refractoriness and patient survival with sex, age, etiology, delay between diagnosis and IST, severity of SAA （onset absolute neutrophil count）, infection at onset, ATG regime, G-CSF therapy and PNH clone in 154 SAA patients treated in Institute of Hematology ＆ Blood Diseases Hospital of Chinese Academy of Medical Science during Dec 1996 to Oct 2006. Results The severity of SAA and infection were risk factors for refractory disease and poor survival. According to multivariate analysis, disease severity and infection were risk factors of IST refractoriness and poor prognosis. Risk of monomer-7 included clonal chromosome evoluting to MDS increased with use of G-CSF for more than 6 months. Conclusion The severity of SAA and occurrence of infection were the major risk factors for refractoriness and poor prognosis of IST, while use of G-CSF for more than 6 months was a risk factor of MDS development.