Evans综合征6例临床分析  

Clinical analysis of 6 cases of Evans syndrome

作　　者：张伟[1] 蒋丽君 王小婕[2] 马燕萍[1] 包慎[1] ZHANG Wei;JIANG Lijun;WANG Xiaojie;MA Yanping;BAO Shen(Department of Hematology,People′s Hospital of Ningxia Hui Autonomous Region,Yinchuan 750002,China;Geriatric Medical Center,Ningxia People′s Hospital of Ningxia Hui Autonomous Region,Yinchuan 750002,China)

机构地区：[1]宁夏回族自治区人民医院血液内科,宁夏银川750002 [2]宁夏回族自治区人民医院老年医学中心,宁夏银川750002

出　　处：《宁夏医学杂志》2021年第3期238-241,共4页Ningxia Medical Journal

基　　金：宁夏回族自治区卫生计生委重点研究课题(2018-NW-032);宁夏回族自治区人民医院培育振兴科研项目(201802)。

摘　　要：目的探讨成人Evans综合征的临床特征。方法对6例成人Evans综合征的临床资料进行回顾性分析。结果6例Evans综合征患者,3例由免疫性血小板减少症(ITP)首发,1例以自身免疫性溶血性贫血(AIHA)首发,2例以Evans综合征起病。4例疑似继发于结缔组织病,1例继发于意义未明的单克隆B淋巴细胞增多症(MBL)。临床主要表现为乏力和出血,其中以AIHA初发者,乏力明显,出血症状轻微;以ITP初发者,出血明显。5例患者Coombs试验阳性,1例阴性。3例以B细胞异常为主,其中1例存在4.6%的单克隆B淋巴细胞。3例以T细胞异常为主,其中1例合并骨髓巨核细胞增生减低。治疗上采取个体化治疗,6例患者获得不同程度的缓解。结论Evans综合征多为基础疾病继发,存在复杂的免疫紊乱,易于复发,治疗困难。在治疗选择上,应依托免疫学、骨髓流式细胞术(FCM)及骨髓病理学等检查结果,采取个体化的治疗,有望提高治疗效果。Objective To investigate the clinical features of Evans syndrome in adults.Methods The clinical data of 6 cases with Evans syndrome were analyzed retrospectively.Results Among the 6 patients with Evans syndrome,3 patients were diagnosed with immune thrombocytopenia(ITP)for the first time,1 patient with autoimmune hemolytic anemia(AIHA)and 2 patients with Evans syndrome.4 cases were suspected to be secondary to connective tissue disease,and 1 case was suspected to be secondary to monoclonal B lymphocytosis(MBL)of unknown significance.The main clinical manifestations were fatigue and bleeding.The patients with initial AIHA had obvious fatigue and mild bleeding symptoms,while those with initial ITP had obvious bleeding symptoms.Coombs test was positive in 5 patients and negative in 1 patient.Among them,1 case had 4.6%monoclone B lymphocytes,3 cases had T cell abnormalities,and 1 case had bone marrow megakaryocyte hyperplasia.In terms of treatment,individualized treatment was adopted,and 6 patients achieved different degrees of remission.Conclusion Evans syndrome is mostly secondary to underlying diseases with complicated immune disorders which is easy to relapse and difficult to treat.In the choice of treatment,we should rely on the results of immunology,bone marrow flow cytometry(FCM)and bone marrow pathology,and take individualized treatment,which is expected to improve the therapeutic effect.

关 键 词：EVANS综合征 免疫性血小板减少症 自身免疫性溶血性贫血 治疗 

分 类 号：R551[医药卫生—血液循环系统疾病]