ALG为主的免疫抑制剂治疗儿童重型再生障碍性贫血的疗效及相关因素

目的通过回顾性分析临床资料,探讨重型再生障碍性贫血(SAA)患儿以抗淋巴细胞球蛋白(ALG)为主的免疫抑制治疗(IST)疗效及相关因素。方法54例重型再生障碍性贫血、并接受ALG治疗患儿的临床资料进行分析。结果基本治愈24例(44.4%),缓解12例(22.2%),明显进步4例(7.4%),无效14例(25.9%),总有效率(74.1%)。典型的血清病多在治疗后7~14 d内出现。随访的54例患儿中1例出现骨髓增生异常综合征(MDS)样病态造血。结论ALG作为重要免疫抑制剂治疗SAA疗效肯定,治疗前患者外周血ANC计数、骨髓涂片中淋巴细胞的比例以及检测CsA有效浓度可能对疗效判断有提示意义。IST后出现重度感染多为预后不良的重要因素之一。

Analysis of the curative effect and its correlation factors of ALG as a Main Regimen of Immunosuppressive Therapy in Treatment of Children Severe Aplastic Anemia

Objective To analyze the effectiveness of antilymphocyte globulin(ALG)in treatment of severe aplastic anemia(SAA).Methods Clinical data of 54 children with severe aplastic anemia who received ATG treatment from March,2003 to January,2007 were analyzed retrospectively.Results Of the 54 patients,24 were nearly cured(44.4%),12 were relieved(22.2%),4 were improved(7.4%)and 14 were invalid(25.9%),The overall effective rate was 74.1%.The effective rate was not related with age,sex,haematopoietic area of bone marrow and the resource ofALG,whereas the patients with neutrophil counts(ANC) more than 0.5 109/L,the percentage of lymphocyte in bone marrow slides less than 70%,the effective concentration of CsA and the relative higher dose of ALG had a lower remission rate(P<0.05).The adverse effects were fever,skin rash,arthralgia,etc,but the typical serum sickness occurred 7~14 d after ALG administration.During the follow-up of 54 patients,1 case occurred myelodysplastic syndrome(MDS).Conclusion ALG in treatment of children severe aplastic anemia was valid and safe.ANC,the percentage of lymphocyte in bone marrow slides and the effective concentration of CsA would be prognostic factors for efficiency in SAA.Severe infection after IST may be the major one of the poor prognostic factors.