儿童自身免疫性溶血性贫血和Evans综合征复发及其相关因素分析

目的研究和探讨儿童自身免疫性溶血性贫血(AIHA)和Evans综合征复发率及其相关因素。方法采用同期病例对照法,比较不同因素与复发的相关性。结果40例患者中有7例复发,复发率为17·5%,Coombs阴性患者复发率为66·66%,Coombs阳性患者复发率为13·51%。复发发生在首次发病的2个月到2年7个月之间,4例在停药后,2例在激素减量中。复发诱因中感染因素占4例(57·14%),主要为呼吸道和消化道感染;无诱因者3例(42·86%)。复发组和未复发组患儿的性别、抗体的类型、是否存在其他自身免疫性疾病、患病时的血红蛋白水平和网织红细胞计数等均与复发无关(P>0·05)。细胞免疫功能的检查结果显示复发组CD3增高、CD8降低,经统计学分析两组存在显著差异(P<0·05)。结论儿童AIHA和Evans综合征的复发率远低于成人,复发患者免疫功能异常较未复发患者显著。

Analysis of the relapse rate and risk factors in children with auto immune hemolytic anemia and Evans syndrome

Objective To analyze the relapse rate and the risk factors of autoimmune hemolytic anemia (AIHA) and Evans syndrome in children. Methods The different risk factors and the relevance of the relapse in 40 cases of AIHA and Evans syndrome were compared by the case-control study. Results There were 7 relapse cases of these AIHA and Evans syndrome in 40 patients. The relapse rate was 17.5%. The relapse rates in patients with negative Coombs test were 66.67%, while there were only 13.51% in the patients with positive Coombs test. The relapse occurred in the period from 2 months to 31 months after the first incidence. 4 cases of relapse occurred after stopping medicine, 2 cases among the period of reducing the steroid. The relapse of the 4 cases was the result of the infection,which was mainly the respiratory infection or the digestive infection,3 cases involved no infection. Among the patients of the relapse group and the non-relapse group, the sex, the types of antibody, immune diseases existed, the hemoglobin level and the numbers of the granulophilocyte in the beginning period of the disease were not relevant to the relapse(p>0.0 5).The results of the examination of cellular immunity showed that CD3 in the relapse group increased, while the CD8 decreased,which was a significant difference between two groups(P<0. 05). Conclusions The relapse rate of AIHA and Evans syndrome in children was much lower than that of adults. The cellular immune abnormality in the relapse patients was greater than that of the non-relapse patients.