血浆置换治疗儿童自身免疫性溶血性贫血疗效分析

Efficacy of plasma exchange in the treatment of autoimmune hemolytic anemia in children

目的了解血浆置换(PE)治疗儿童自身免疫性溶血性贫血(AHA)的有效性及安全性。方法收集2014年11月至2017年4月国内8家医院行PE治疗AHA患儿的临床资料, 对其临床特点进行回顾性分析。结果共21例AHA患儿纳入统计, 17例来自PICU, 4例来自小儿肾脏病房。男11例, 女10例, 中位年龄3.64(0.25, 11.10)岁, 中位住院时间12(4, 45)d。感染相关15例(71.4%), 自身免疫性疾病2例(9.5%), 病因不详4例(19.0%)。PE前意识障碍4例, 置换后均恢复正常;血容量减少2例(9.5%), 置换后均恢复正常;贫血20例(95.2%), 置换后15例正常, 5例好转;黄疸18例(85.7%), 置换后12例正常, 6例好转;肝脾肿大11例, 置换后10例正常, 1例好转。PE后患儿血红蛋白、红细胞计数较前升高, 总胆红素、间接胆红素、尿素氮、乳酸脱氢酶较PE前下降, 差异均有统计学意义(P均<0.05)。仅1例患儿出现过敏反应, 予对症处理后好转, 继续PE操作。PE治疗后完全缓解2例(9.5%), 部分缓解16例(76.2%), 退院3例(14.3%)。结论 PE治疗可明显改善经药物保守治疗无效的AHA患儿临床症状及实验室指标, 可作为重症AHA患儿的救治措施, 有较好的安全性。

Objective To investigate the efficacy and safety of plasma exchange(PE)in the treatment of autoimmune hemolytic anemia in children.Methods The data from 8 hospitals in China during November 2014 to April 2017 were collected,and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results A total of 21 children with AHA were included in the study,including 17 cases from PICU and 4 cases from pediatric kidney ward,with 11 boys and 10 girls,and the median age was 3.64(0.25,11.10)years old,and median hospital stay was 12(4,45)days.There were 15 cases(71.4%)with infection,2 cases(9.5%)with autoimmune diseases,4 cases(19.0%)with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%)and completely relieved.There were 20 cases of anemia(95.2%),15 cases were normal after PE,and 5 cases were improved.Jaundice occurred in 18 cases(85.7%),12 cases were normal after PE,6 cases were improved.Hepatosplenomegaly was found in 11 cases,10 cases were normal after PE,1 case was improved.After PE,the hemoglobin and red blood cell count increased,while the total bilirubin,indirect bilirubin,urea nitrogen and lactate dehydrogenase decreased,there were significant differences between pre-and post-replacement(P<0.05).Only 1 case had allergic reaction,which was improved after symptomatic treatment,and PE was continued.After PE,2 cases(9.5%)had complete remission,16 cases(76.2%)had partial remission and 3 cases(14.3%)had been discharged.Conclusion PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.