儿童肝炎相关再生障碍性贫血五例临床特点  

Clinical features of five children with hepatitis-associated aplastic anaemia

作　　者：王美娟[1] 钟雪梅[1] 马昕[1] 宁慧娟[1] 朱丹[1] 宫幼喆[1] 刘嵘[2] Wang Meijuan;Zhong Xuemei;Ma Xin;Ning Huijuan;Zhu Dan;Gong Youzhe;Liu Rong(Department of Gastroenterology,Children′s Hospital,Capital Institute of Pediatrics,Beijing 100020,China;Department of Hematology,Children′s Hospital,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院消化内科,北京100020 [2]首都儿科研究所附属儿童医院血液内科,北京100020

出　　处：《中国小儿急救医学》2022年第12期994-997,共4页Chinese Pediatric Emergency Medicine

摘　　要：目的探讨儿童肝炎相关再生障碍性贫血(HAAA)的临床特征,以期早期诊断及治疗。方法回顾性分析2016年1月至2020年12月首都儿科研究所附属儿童医院消化内科收治的5例以急性肝功能异常入院后经骨髓检查确诊再生障碍性贫血(AA)患儿的临床表现、实验室检查、治疗等临床资料。结果5例患儿均为男童,发病年龄2~13岁,5例患儿肝炎均为急性起病,均有皮肤黄染症状,肝炎至诊断AA的间隔时间为0~12周,其中1例患儿肝炎及AA同期发作,3例患儿诊断AA时肝功能明显好转,1例患儿诊断AA时转氨酶再次明显升高。5例患儿中仅1例患儿血清巨细胞病毒DNA阳性,余病原学检测均阴性。5例患儿均存在淋巴细胞免疫紊乱,抑制/细胞毒性T淋巴细胞(CD3^(+)CD8^(+))比例升高。2例患儿接受造血干细胞移植治疗,其中1例移植后死亡,1例好转;其余3例患儿中1例患儿予抗胸腺细胞免疫球蛋白及环孢素治疗后好转,1例患儿早期因重症感染死亡,1例患儿予环孢素治疗无明显好转。结论临床上在治疗急性肝炎患儿时,需警惕发生HAAA的可能性,即使肝功能好转,仍需监测血常规变化。当外周血2系或3系减低时,需积极完善骨髓检查,早期及时予免疫抑制剂及骨髓造血干细胞移植治疗可改善患儿预后。Objective To investigate the clinical characteristics of hepatitis-associated aplastic anaemia(HAAA)in children.Methods A retrospective analysis was performed on the clinical manifestations,laboratory examinations,treatments and other clinical data of five children with aplastic anemia(AA)diagnosed by bone marrow examination after admission with acute liver dysfunction admitted to the Department of Gastroenterology,Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2016 to December 2020.Results All five children were boys and the onset age of these children ranged from 2 to 13 years.All of the five cases were acute onset and presented with jaundice.The time frame of the diagnosis of HAAA was 0 to 12 weeks from the presentation of the liver disease.One patient had simultaneous onset of hepatitis and aplastic anemia.The liver function was significantly improved at the diagnosis of HAAA in three patients and worsen in one patient.Only one patient showed CMV-DNA positive and the pathogen results of other patients were negative.Lymphocyte immunity disorders were found in all five patients,and the proportion of inhibitory/cytotoxic T lymphocytes(CD3^(+)CD8^(+))increased.Two children received hematopoietic stem cell transplantation,of which one died and one improved after transplantation.One child improved after treated with antithymocyteglobulin and cyclosporin.One child died due to severe infection.There was no significant improvement in one child treated with cyclosporine.Conclusion HAAA should be alerted in acute hepatitis patients.Blood routine should be monitored even if liver function improves.Bone marrow tests are needed if patients have peripheral cytopenia in two or more lineages.Early and timely treatments with immunosuppressive therapy and hematopoietic stem cell transplantation can improve the prognosis.

关 键 词：肝炎 再生障碍性贫血 临床特征 儿童 

分 类 号：R725.7[医药卫生—儿科] R725.5[医药卫生—临床医学]