19例儿童及青少年Evans综合征回顾性临床分析  

A retrospective study of 19 childhood and adolescent patients with Evans syndrome in a single center

作　　者：董恂玮[1] 赵玉平[1] 郑以州[1] 张凤奎[1] 杨仁池[1] 

机构地区：[1]中国医学科学院北京协和医学院血液病医院(血液学研究所),天津300020

出　　处：《临床血液学杂志》2011年第1期28-29,32,共3页Journal of Clinical Hematology

基　　金：公益性卫生行业科研专项基金(No:200802031)

摘　　要：目的:评价联合治疗对儿童及青少年Evans综合征的疗效。方法:回顾性分析1984-2007年19例儿童及青少年Evans综合征患者的临床特征以及各种治疗方案的疗效。结果:19例患者中17例女性,2例男性,所有患者均给予标准或大剂量糖皮质激素作为一线治疗,完全缓解率达64.29%,中位随访24个月,88.89%患者复发。15例患者给予了二线治疗,完全缓解及部分缓解率达到86.67%。4例患者行脾切除,其中3例达完全缓解,但在中位6个月内2例复发。结论:鉴于Evans综合征难治易复发的特点,我们建议联合治疗可取得较好的效果,药物治疗失败后可考虑脾切除。Objective：To evaluate the efficacy of combination treatments for childhood and adolescent patients with Evans syndrome.Method：In this retrospective study,we evaluated the clinical features and the effects of various treatment regimens on the clinical course of childhood and adolescent patients with Evans syndrome in our center between 1984-2007.Result：Nineteen （17 females,2 males） patients were included. All patients were treated with either standard or high-dose steroids as first line therapy. CR was achieved in 64.29% patients with steroids as the initial therapy. During a median 24 months follow-up,relapse occurred in 88.89% patients. Fifteen patients were treated with second-line therapies. CR and PR were obtained in 86.67% patients. Splenectomy was performed in 4 patients and CR was obtained in 3 of them. Relapse occurred within median 6 months in 66.67% patients who had CR.Conclusion：In view of the refractory,persistent nature of Evans syndrome,we suggest that treatment with combination agents may provide a useful therapeutic approach. Splenectomy may be considered after drug therapy failed.

关 键 词：EVANS综合征 一线治疗 二线治疗 脾切除 免疫球蛋白 免疫失调 

分 类 号：R556.6[医药卫生—血液循环系统疾病]