肝炎相关性再生障碍性贫血合并噬血细胞综合征1例并文献复习  

A case of hepatitis-associated aplastic anemia complicated by hemophagocytic lymphohistiocytosis and literature review

作　　者：周鑫 陈晓宇 文川[1] 罗森林[1] ZHOU Xin;CHEN Xiao-Yu;WEN Chuan;LUO Sen-Lin(Department of Pediatrics,Second Xiangya Hospital of Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院儿童医学中心,湖南长沙410011

出　　处：《中国当代儿科杂志》2025年第4期465-471,共7页Chinese Journal of Contemporary Pediatrics

摘　　要：患儿,男,4岁,因皮疹、间断腹痛3 d,发现血常规结果异常半天入院。初期以急性黄疸型肝炎和全血细胞减少为主要表现。患儿病情进展迅速,入院后出现反复发热,皮肤及巩膜黄染加重,肝脾大且进行性加重,肝功能损伤及骨髓衰竭程度持续恶化,细胞因子水平持续上升,排除了感染、自身免疫性疾病、肿瘤、遗传代谢性疾病及中毒等疾病,该患儿诊断为肝炎相关性再生障碍性贫血(hepatitis-associated aplastic anemia,HAAA)合并噬血细胞综合征(hemophagocytic lymphohistiocytosis,HLH)。经过糖皮质激素、血浆置换、静脉注射免疫球蛋白及护肝治疗,患儿症状得到部分缓解。再生障碍性贫血合并HLH较为少见,尤其是HAAA合并HLH,更为罕见、隐匿及凶险。该文报道1例HAAA合并HLH的病例,并对文献已报道的病例资料进行归纳总结,为该病的早期诊断、治疗提供参考。A 4-year-old boy was admitted to the hospital with a 3-day history of rash and intermittent abdominal pain,during which abnormal results from routine blood tests were discovered.Initially,he presented with acute jaundice hepatitis and pancytopenia.The patient's condition progressed rapidly,with recurrent fever,worsening jaundice of the skin and sclera,and progressively worsening hepatosplenomegaly.Liver function impairment and bone marrow failure continued to deteriorate,while cytokine levels continued to rise.After excluding infections,autoimmune diseases,tumors,genetic metabolic disorders,and toxicities,the patient was diagnosed with hepatitis-associated aplastic anemia(HAAA)complicated by hemophagocytic lymphohistiocytosis(HLH).Following treatment with corticosteroids,plasma exchange,intravenous immunoglobulin,and liver protection therapy,the patient's symptoms partially alleviated.Aplastic anemia complicated by HLH is relatively uncommon,and HAAA complicated by HLH is even rarer,often presenting insidiously and severely.This paper presents a case of HAAA complicated by HLH and summarizes previously reported cases in the literature,providing references for the early diagnosis and treatment of this condition.

关 键 词：肝炎相关性再生障碍性贫血 噬血细胞综合征 儿童 

分 类 号：R725.5[医药卫生—儿科]