儿童肝炎相关再生障碍性贫血11例并文献复习  

Hepatitis-associated aplastic anemia in children: report of 11 cases and literature review

作　　者：凌业生 罗湘琴 李晓峰[1] 陈惠芹[1] Ling Yesheng;Luo Xiangqin;Li Xiaofeng;Chen Huiqin(Department of Pediatrics, the Third Affiliated Hospital of Sun Yat-senUniversity, Guangzhou 510630, China)

机构地区：[1]中山大学附属第三医院儿科

出　　处：《新医学》2019年第10期781-787,共7页Journal of New Medicine

摘　　要：目的探讨儿童肝炎相关再生障碍性贫血(HAAA)的临床特点,加深对该病的认识,避免误诊。方法对11例HAAA患儿的临床资料进行回顾性分析,并以“肝炎相关再生障碍性贫血/hepatitis associated aplastic anemia”为检索词在中国期刊全文数据库(CNKI)、万方数据知识服务平台及PubMed收集并分析相关文献。结果儿童HAAA发病年龄以学龄期为主,男性多于女性,多数患儿起病时无明显诱因出现非甲、乙、丙、丁、戊型病毒性肝炎,不除外与巨细胞病毒、Epstein-Bar病毒、单纯疱疹病毒等病毒感染有关;肝损害以血清转氨酶升高为主,伴或不伴血清总胆红素升高,再生障碍性贫血(AA)可同时或晚于肝炎出现,AA包括特发性再生障碍性贫血和免疫性全血细胞减少。免疫学检查方面:自身免疫性肝炎抗体可为阴性,包括抗血小板抗体、抗核抗体等多种非特异性抗体可为阳性,淋巴细胞亚群比例异常,HAAA患儿使用静注人免疫球蛋白、糖皮质激素、抗胸腺细胞免疫球蛋白、环孢素等免疫抑制治疗部分有效,但需警惕感染,如肺部感染。结论儿童HAAA相对少见,病因未明确,与免疫介导T淋巴细胞功能紊乱相关,一旦临床诊断,除了积极治疗肝炎外,应尽早给予患儿以免疫抑制治疗为主的综合治疗。Objective To investigate the clinical characteristics of pediatric hepatitis-associated aplastic anemia (HAAA), aiming to deepen the understanding of HAAA and avert the risk of misdiagnosis. Methods Clinical data of 11 children diagnosed with HAAA were retrospectively analyzed. Literature review was conducted from CNKI, Wanfang Data and PubMed by using the keywords of hepatitis-associated aplastic anemia in both Chinese and English. Results Pediatric HAAA predominantly occurred in children of the school-age with a higher prevalence in boys than girls. A majority of the affected children developed hepatitis with unknown causes (non-A,-B,-C,-D or -E hepatitis) during the onset of the disease. The possible association with cytomegalovirus, Epstein-Bar virus and herpes simplex virus infection could not be excluded. Liver function damage was mainly characterized by elevated serum transaminase, complicated with or without total bilirubin elevation. Aplastic anemia (AA) might occur simultaneously or later than hepatitis. AA consisted of idiopathic aplastic anemia and immunorelated pancytopenia. Immunological examination demonstrated that autoimmune hepatitis antibodies were negative, whereas multiple non-specific antibodies including anti- platelet antibody, anti-nuclear antibody were positive. The proportion of lymphocyte subgroups was abnormal. Partial children with HAAA were effectively treated after intravenous immunoglobulin, glucocorticoid, anti- thymocyte immunoglobulin, ciclosporin and other immunosuppressive agents. However, the possibility of infection, such as pulmonary infection, should be cautious. Conclusions Pediatric HAAA is relatively rare in clinical practice and the pathogenesis remains elusive. It is correlated with immunity-mediated T lymphocyte dysfunction. If the child is clinically diagnosed with HAAA, immunosuppressive agents-based comprehensive treatment along with active treatment of hepatitis should be delivered immediately.

关 键 词：肝炎相关再生障碍性贫血 儿童 临床特点 诊断 治疗 

分 类 号：R725.5[医药卫生—儿科]