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抗胸腺细胞/抗淋巴细胞球蛋白对重型再生障碍性贫血及治疗相关并发症的治疗作用

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

作者信息

Yu Fang, Tang Suo-Qin, Wang Jian-Wen

机构信息

Department of Pediatrics, General Hospital of People's Liberation Army, Beijing 100853, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2006 Dec;8(6):479-81.

Abstract

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.

摘要

目的

免疫抑制治疗是重型再生障碍性贫血的主要治疗方法,通常使用抗胸腺细胞/抗淋巴细胞球蛋白(ATG/ALG)。本研究探讨了ATG/ALG治疗重型再生障碍性贫血的疗效,并探索了治疗相关并发症的处理方法。

方法

回顾性分析1994年12月至2005年9月期间接受ATG/ALG治疗的28例重型再生障碍性贫血患儿的临床资料。

结果

根据血红蛋白/白细胞/血小板计数,28例患者中2例基本治愈(7.1%),4例缓解(14.3%),12例改善(42.9%)。总有效率为64.3%。19例患者出现血清病的临床证据,表现为发热(9例)、皮疹(12例)、关节痛(7例)、肌痛(7例)和关节肿胀(3例)。血清病发生在ATG/ALG给药后5 - 17天,持续1 - 15天(平均4.4天)。3例血清病症状较轻的患儿未经任何治疗即康复。其他16例患者在甲泼尼龙治疗(每日10 mg/kg)3 - 5天后症状消失。然而,3例患者在甲泼尼龙治疗终止后2 - 4天复发。对这3例患者再次给予甲泼尼龙治疗,直至症状消失。无血清病或血清病症状较轻的患者对ATG/ALG治疗的反应优于血清病严重的患者(100%对60%;P < 0.05)。

结论

ATG/ALG治疗重型再生障碍性贫血有效。血清病是重型再生障碍性贫血患儿接受ATG/ALG治疗后常见的并发症,但应用甲泼尼龙可改善。

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