Evans综合征8例病例分析  

Analysis 8 Case of EVANS Syndrome

作　　者：李红杰 王强[2] LI Hong-＇jie,WANG Qiang （1. Dingzhou City TCM Hospital in Hebei Province,Dingzhou 073000,China; 2.Beijing Xiehe Hospital,Chinese Medical Science Insistute,Beijing 100730,China ）

机构地区：[1]河北省定州市中医医院内科,河北定州073000 [2]中国医学科学院北京协和医院内科,100730

出　　处：《医学信息》2011年第21期79-81,共3页Journal of Medical Information

摘　　要：目的通过总结既往诊断的Evans综合征病例特点、病因及治疗反应、转归．提高对Evans综合征的认识。对象与方法回顾性分析河北省定州市中医医院1990年一20t0年10月确诊的Evans综合征8例。并进行国内外相关文献复习。结果①一般特点：男3例，女5例，平均诊断年龄35岁，确诊时平均病程55年。②临床特征：6例因感染、药物诱发加重，1例继发于SLE，2例可能继发于免疫病；均有贫血、黄疸，7例有出血倾向，3例有酱油色尿，6例脾轻中度大，2例肝轻度大；均有HGB及PLT减低，血LDH及TBIL增高，IBIL为主，骨穿符合ITP及溶贫，血Ig升高（3／8），Coombs试验阳性（6／8），PAIgG增高（6／6），ANA〉1：80阳性（3／8），补体减低（6／7）。③治疗及效果：采用激素（8／8）、迭那唑（3／8）及免疫抑制荆（5／8）治疗，2例完全缓解，4例部分缓解，1例无效，1例自动出院。Objective To improve the understanding of Evans syndrome,we analyze the clinical features, etiology and treatment of Evans syndrome. Methods Retrospectively analyze the clinical data of 8 cases with Evans syndrome in Chinese Medicine Hospital of Ding Zhou,Heibei Province from 1990 to October 2010, and review the related literatures. Results ①common features： three were men, and five were women. The median age at diagnosis was 35 years. The average course of disease was 5.5 years before diagnosis. ②clinical features： six of the eight patients were induced by infections and drugs, one was secondary to SLE, and two may secondary to autoimmune disease. All patients had anemia and jaundice, seven had the tendency of bleeding, six had splenomegaly, three had saucy urine, and two had hepatomegaly. All had decreased HGB and PLT, increased LDH and TBIL （IBIL mainly）; and other features including increased immunoglobulin （3/8）, Coombs test （＋）（6/8）,increased PAIgG （6/6）, ANA〉I：80 （＋）（3/8）, and decreased complement （6/7）. ③treatment and results： the patients accepted the therapy of glucocorticoid （8/8）, danazol （3/8） and immunosupprossant （5/8）, 2/7 got CR, 417 got PR, and 1/7 got no effect. Conclusion Evans syndrome, which is rare in clinic, is less likely to respond well to therapy, and is tend to recur. The internist should attach importance to it and treat it with an active way.

关 键 词：EVANS综合征 自身免疫性溶血性贫血(AIHA) 免疫性血小板减少性紫瘢(ITP) 

分 类 号：R556.6[医药卫生—血液循环系统疾病;医药卫生—临床医学;医药卫生—内科学]