儿童单倍体造血干细胞移植后并发3例自身免疫性溶血性贫血的临床探讨  

Clinical study of 3 cases of autoimmune hemolytic anemia after haploidentical hematopoietic stem cell transplantation in children

作　　者：李洁[1] 陈凯[1] 张娜[1] 刘青[1] 邹冰 邵静波[1] 李红[1] 朱嘉莳[1] 蒋慧[1] LI Jie;CHEN Kai;ZHANG Na;LIU Qing;ZOU Bing;SHAO Jingbo;LI Hong;ZHU Jiashi;JIANG Hui(Department of Hematology and Oncology,Shanghai Children′s Hospital,Children′s Hospital Affiliated to Shanghai Jiaotong University,Shanghai 200040,China)

机构地区：[1]上海市儿童医院,上海交通大学附属儿童医院血液肿瘤科,上海200040

出　　处：《中国小儿血液与肿瘤杂志》2022年第2期106-109,共4页Journal of China Pediatric Blood and Cancer

基　　金：上海市卫生健康委员会科研课题基金(20194Y0112);上海申康医院发展中心重大临床研究项目(SHDC2020CR4089)。

摘　　要：目的探讨儿童单倍体造血干细胞移植(Haplo-HSCT)后并发自身免疫性溶血性贫血(AIHA)的临床特征。方法回顾性分析本中心Haplo-HSCT后AIHA发生时间、临床表现、治疗及转归。结果60例Haplo-HSCT治疗后患儿中有3例(5%)出现AIHA,原发病均为再生障碍性贫血。干细胞来源为外周血造血干细胞,接受了减低强度预处理。3例供受者血型不合(2例主要不合,1例次要不合),移植前输血次数均大于10次,移植后并发不同病毒感染。经综合治疗后(糖皮质激素、免疫抑制剂、丙种球蛋白及利妥昔单抗等)溶血均被控制,其中病例3在初次溶血控制后再次反复,多种二线药物治疗无效,最终经西罗莫司联合小剂量糖皮质激素治疗后痊愈。结论造血干细胞移植后AIHA是严重移植并发症之一,多药联合及时有效治疗可改善移植最终预后。Objective To explore the clinical characteristics of autoimmune hemolytic anemia(AIHA)after haploidentical hematopoietic stem cell transplantation(Haplo-HSCT)in children.Methods A series of clinical data including the onset time,manifestation,treatment and prognosis of 3 cases of AIHA after Haplo-HSCT were analyzed retrospectively.Results AIHA occurred in 3 of 60 Haplo-HSCT patients(5%).The primary diseases of these 3 children were all aplastic anemia,and stem cells were derived from peripheral blood stem cells.All of them received reduced-intensity conditioning regimen.The donor and recipient blood groups were all incompatible(2 cases were major incompatible and 1 case was minor incompatible).The times of blood transfusion before transplantation were all more than 10 times.They all had virus infection after transplantation.AIHA could be controlled by combined treatment including corticosteroids,immunosuppressant,intravenous immunoglobulin,rituximab,etc.In case 3,the hemolysis was repeated and was not controlled by a variety of second-line drugs.And then after sirolimus combined with low-dose corticosteroids treatment,the hemolysis was gradually controlled.Conclusions AIHA after HSCT is one of the serious complications of transplantation.Multi-drug combination and timely and effective treatment can improve the final prognosis.

关 键 词：儿童 单倍体造血干细胞移植 自身免疫性溶血性贫血 治疗 

分 类 号：R55[医药卫生—血液循环系统疾病]