联合免疫抑制剂疗儿童重型再生障碍性贫血临床研究.doc

联合免疫抑制剂治疗儿童重型再生障碍性贫血临床研究 王高燕 李学荣 孙立荣 仲任 赵艳霞 卢愿 宋爱琴 庞秀英 青岛大学医学院附属医院小儿血液科（山东青岛266003） 【摘要】目的观察抗胸腺细胞免疫球蛋白（ATG）、环孢素（CsA）和大剂量丙种球蛋白（HDIVIG）联合免疫抑制治疗儿童重型再生障碍性贫血（SAA）的疗效，并评价其临床安全性。方法选取2004年5月—2009年3月确诊并采用ATG＋CsA＋HDIVIG联合治疗的SAA儿童20例，对其临床资料进行分析。结果ATG＋CsA＋HDIVIG联合治疗儿童SAA有效率和显效率分别为80％（16／20）和70％（14／20），20例中基本治愈11例，缓解3例，进步2例，无效4例（其中死亡2例）。ATG用药期间出现过敏反应2例，治疗相关鼻衄5例，其中2例同时合并肉眼血尿。治疗过程中17例患儿发生不同程度感染，发生血清病反应2例，继发性糖尿病5例，高血压7例，低蛋白血症8例。随访期内未发现克隆性疾病。结论联合免疫抑制剂（ATG＋CsA＋HDIVIG）是治疗儿童SAA的一种安全有效的治疗方法。 【关键词】联合免疫抑制；再生障碍性贫血；治疗；抗胸腺细胞免疫球蛋白；环孢素；丙种球蛋白 Clinical research of combined immunosuppressive therapy on severe aplastic anemia in children WANG Gao-yan，LI Xue-rong，SUN Li-rong，ZHONG Ren，ZHAO Yan-xia，LU Yuan，SONG Ai-qin，PANG Xiu-ying（Department of Pediatric Hematology，Hospital Affiliated to Medical College，Qingdao University，Qingdao 266003，Shandong，China） Abstract：Objective Clinical therapeutic effects of combined immunosuppressive（CIS）therapy with antithymocyte globulin（ATG），cyclosporine A（CsA）and high dose of intravenous immunoglobulin（IVIG）on severe aplastic anemia（SAA）in children was studied and its clinical safety was evaluated．Methods Retrospective analysis was performed on 20 patients diagnosed as SAA who were treated by ATG＋CsA＋high dose IVIG from May 2004 to March 2009．Results The therapeutic effective rate was 80％，and significant effective rate was 70％．Among 20 cases，11 cases recovered well on the whole，3 were relieved，2 were improved，4 were ineffective（two of them died）．During the procedure of treatment，anaphylaxis appeared in two cases，epistaxis in five cases，two of them accompanied with macroscopic hematuria，infection appeared in 17 cases，serum sickness reaction in two cases，and five cases developed secondary diabetes mellitus，seven cases had hypertension，eight cases had hypoproteinemia．Clonal myeloproliferative disease had not been found during follow-up．Conclusions Combined immunosuppressive therapy with ATG＋CsA＋high dose IVIG on SAA in children is an effective and safe med