目的 分析不同剂量的静脉丙种球蛋白(IVIG)辅助治疗儿童获得性重型再生障碍性贫血(AA)的疗效.方法 回顾分析2000年1月至2015年12月应用IVIG辅助免疫抑制治疗的获得性重型AA住院患儿的临床资料.并根据治疗情况分为低剂量组,IVIG 200~400 mg/(kg·d),每4周一次,连用6次;高剂量组,IVIG 1 g/(kg·d),连用2天,每4周1次,连用6次.结果 所有患儿随访至2015年12月31日,61例患儿中41例治疗有效,总有效率为67.2 %.高剂量组在抗胸腺细胞球蛋白(ATG)治疗后3个月的有效率高于低剂量组,差异有统计学意义(P=0.020).20例无效患儿IVIG首剂应用距离ATG首剂间隔时间为2.0 d(2.0~5.0 d),而41例有效患儿间隔时间为8 d(7.0~9.0 d),两组间比较差异有统计学意义(P<0.001);在20例无效患儿中有18例ATG与IVIG的使用时间间隔<7 d.两组生存率分别为80.0 %和87.1 %,差异无统计学意义(P>0.05).两组患儿应用抗胸腺细胞球蛋白(ATG)后6个月内,高剂量组严重感染的发生率低于低剂量组,差异有统计学意义(P=0.008).结论 应用免疫抑制治疗的获得性重型AA患儿,加用高剂量IVIG辅助治疗可使早期反应率增加,但并未增加其远期有效率、治愈率及5年生存率;可减少严重感染率,但未能减少总感染率及感染相关死亡率.%Objective To analyze the efficacy of different doses of intravenous immunoglobulin (IVIG) in the treatment of acquired severe aplastic anemia (AA) in children. Methods The clinical data of hospitalized children with severe AA who received adjuvant immunosuppressive therapy of IVIG from January 2000 to December 2015 were retrospectively analyzed. According to different doses of treatment, the children were divided into low dose group ( IVIG 200-400 mg/ (kg·d) once every 4 weeks for 6 times), high dose group (IVIG 1 g/ (kg·d ) x 2 days once every 4 weeks for 6 times). Results All the children were followed up until December 31, 2015. Among the 61 children, it was effective in 41 children and total effective rate was 67.2%. The effective rate of anti thymocyte globulin (ATG) treatment in high dose group was higher after 3 months than that of low dose group, and there was statistical difference (P=0.020). The interval between first dose of IVIG and first dose of ATG in 20 cases of ineffectiveness was 2.0 (2.0-5.0) d, while that in 41 cases of effectiveness was 8.0 (7.0-9.0) d, and the difference is statistically significant (P<0.001); Among the 20 ineffective children, 18 children had the interval <7 day. The survival rates of the two groups were 80% and 87.1%, respectively, and there was no difference between two groups (P>0.05). The incidence of severe infections in the high-dose group was lower than that in the low-dose group after the use of ATG for 6 months, and there was statistical difference (P=0.008). Conclusions High dose of IVIG therapy can increase the early response rate in children with acquired severe AA, but it does not increase the long-term effectiveness, cure rate and 5 year survival rate. In addition, it can reduce the severe infection rate, but cannot reduce the total infection rate and infection related mortality rate.
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