环胞菌素A治疗儿童再生障碍性贫血疗效研究

STUDY ON CYCLOSPORIN A (csa) IN THE TREATMENT OF CHILDHOOD APLASTIC ANEMIA

探讨环胞菌素 A(CSA)治疗儿童再生障碍性贫血 (再障 )的方法 ,疗效和疗效相关因素。方法 :应用 CSA对 34例儿童再障行免疫抑制治疗 (IS) ;部分重型再障(SAA)加用抗胸腺细胞球蛋白 (ATG)或大剂量免疫球蛋白 (HDIG) ,均以雄性激素作为辅助治疗。结果 :基本治愈 4例 ,缓解 1 1例 ,明显进步 8例 ,总有效率为 6 7.6 5 % ;其中 2 9例 SAA总有效率为 6 5 .5 2 % ,1 8例慢性重型再障 (SAA- )有效率为5 5 .5 6 % ;1 1例急性再障 (SAA- )总有效率达 81 .82 % ,单项资料对比分析结果显示SAA的年龄 ,性别和治疗前外周血象等因素与 CSA有效率无关 ;但病程较短者 (<1 2个月 )有效率较高 ;CSA与 ATG等行联合 IS则有效率可明显提高。结论 :CSA为治疗儿童再障的有效方法之一 ,SAA拟选用联合 IS,雄性激素为

To evaluate the theraputic methods, clinical efficacy and outcone relative factors of cyclosporin A (CSA) in the treatment of childhood aplastic anemia. methods:34 cases of chilidhood patients were treated with CSA. Part of the patients with severe aplastic anemia (SAA) were treated with combination of CSA and antithymocyte globulin (ATG) and/or high dose immunoglobulin (HDIG). Results:The overall resopnse rate was achieved to 67.65%(23/34) including cure on 4 cases, complete respones on 11 cases and partial response on 8 cases. There were 29 cases of SAA in the study group (11 cases of SAA I and 18 cases of SAA Ⅱ),the response rate of SAA Ⅰ and SAA Ⅱ were 81.82% and 55.56% respectively. Factors predictive of response study showed no statistical influence to repsonse rate in patient's age, sex and pretreatment peripheral blood counts. The patients whohad their disease for <121 months achieved response rate of 83.33% compared with 52.99% if disease was of >12 months duration (P<0,05) The response rate in patients treated with combination of CSA and ATG and/or HDIG were significantly higher than that with CSA alone.Conclusion:CSA was onc of the effective immunosuppressive(IS) agents for childhood aplastic anemia. Combination of IS therapy may be more suitable to SAA, and androgens were of benefits to IS therapy.