抗胸腺球蛋白/抗淋巴细胞球蛋白治疗重型再生障碍性贫血的疗效及其并发症

目的：目前重型再生障碍性贫血的主要治疗手段之一是免疫抑制治疗,而抗胸腺球蛋白/抗淋巴细胞球蛋白(ATG/ALG)是其中重要的药物。该文通过回顾性分析临床资料,探讨ATG/ALG治疗重型再生障碍性贫血的疗效及其并发症的防治。方法：对1994年12月至2005年9月收治的28例诊断为重型再生障碍性贫血并接受ATG/ALG治疗的患儿的临床资料进行分析。结果：28例患儿中,基本治愈2例(7.1%),缓解4例(14.3%),进步12例(42.9%),总有效率64.3%。19例出现血清病样反应,主要表现发热9例,皮疹12例,关节痛7例,肌肉痛7例,关节肿胀3例。发生时间为用药后5~17d,持续时间1~15d,平均4.4d。3例轻症者未经处理自行缓解。其余16例给予甲基泼尼松龙每日10mg/kg,静脉推注每天1次,连用3~5d,症状均消失。3例于停用甲基泼尼松龙2~4d后再次出现血清病症状,再次给予甲基泼尼松龙后症状消失。无血清病及血清病反应轻微者ATG/ALG疗效明显优于血清病反应重者(P<0.05)。结论：ATG/ALG治疗重型再生障碍性贫血疗效肯定,血清病为治疗中常见的不良反应,应用甲基泼尼松龙3~5d可较好控制血清病症状。

Therapeutic effect of antithymocyte/antilymphocyte globulin on severe aplastic anemia and therapy-related complications

Objective Immunsuppressive therapy is a major therapy for severe aplastic anemia, and antithymocyte /antilymphocyte globulin (ATG/ALG) is usually used. This study investigated the therapeutic effect of ATG/ALG on severe aplastic anemia and explored the management of therapy-related complications. Methods Clinical data of 28 children with severe aplastic anemia who received ATG/ALG treatment from December, 1994 through to September, 2005 were analyzed retrospectively. Results Of the 28 patients, 2 were nearly cured (7.1%), 4 were relieved (14.3%) and 12 were improved (42.9%) based on a hemoglobin/white blood cell/platelet count. These results represented an overall effective rate of 64.3%. Clinical evidence of serum sickness developed in 19 patients, manifesting as fever (n=9), cutaneous eruptions (n=12), arthralgias (n=7), myalgia (n=7) and arthrocele (n=3). Serum sickness occurred 5-17 days after ATG/ALG administration and lasted for 1-15 days (mean 4.4 days). Three children with mild serum sickness symptoms recovered without any treatment. The symptoms of the other 16 patients disappeared after 3-5 days of methylprednisolone treatment (10 mg/kg daily). However, 3 patients had relapses at 2-4 days after termination of methylprednisolone therapy. Another course of methylprednisolone therapy was administered to the 3 patients until the symptoms disappeared. The patients with no serum sickness or with mild serum sickness had a better response to ATG/ALG therapy than those who had severe serum sickness (100% vs 60%; P<0.05).Conclusions ATG/ALG therapy for severe aplastic anemia is effective. Serum sickness is a common complication in children with severe aplastic anemia following ATG/ALG therapy, but can be improved by methylprednisolone application.