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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 for poor prognosis in patients with severe aplastic anemia (SAA) refractory to combined immunosuppressive therapy (IST). Methods The multivariate analysis was used to review the gender, age, etiology, time from diagnosis to IST, severity of disease (onset of neutrophilic granulocyte at onset) from December 1996 to October 2006 in 154 patients with SAA at the Hematology Hospital of Chinese Academy of Medical Sciences ), Infection at onset, anti-thymocyte globulin (ATG) dosage form, application of granulocyte colony-stimulating factor (G-CSF) and paroxysmal nocturnal hemoglobinuria (PNH) And the relevance of survival. Results Multivariate analysis showed that the severity of the disease and infection were risk factors for refractory and poor prognosis of patients with SAA. Application of G-CSF> 6 months Increased risk of clonal chromosome transformation including monomer 7, ie, myelodysplastic syndrome (MDS). Conclusion The severity of the disease and the occurrence of infection are the main risk factors for refractory and poor prognosis of patients with SAA. G-CSF application time greater than 6 months is a risk factor for MDS conversion.