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[1例合并噬血细胞性淋巴组织细胞增生症的肝炎相关性再生障碍性贫血病例及文献复习]

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

作者信息

Zhou Xin, Chen Xiao-Yu, Wen Chuan, Luo Sen-Lin

机构信息

Department of Pediatrics, Second Xiangya Hospital of Central South University, Changsha 410011, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2025 Apr 15;27(4):465-471. doi: 10.7499/j.issn.1008-8830.2409118.

Abstract

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.

摘要

一名4岁男孩因皮疹和间歇性腹痛3天入院,在此期间发现常规血液检查结果异常。最初,他表现为急性黄疸型肝炎和全血细胞减少。患者病情迅速进展,反复发热,皮肤和巩膜黄疸加重,肝脾肿大逐渐恶化。肝功能损害和骨髓衰竭持续恶化,而细胞因子水平持续升高。在排除感染、自身免疫性疾病、肿瘤、遗传代谢性疾病和中毒后,患者被诊断为肝炎相关性再生障碍性贫血(HAAA)合并噬血细胞性淋巴组织细胞增生症(HLH)。经过糖皮质激素、血浆置换、静脉注射免疫球蛋白和保肝治疗后,患者症状部分缓解。再生障碍性贫血合并HLH相对少见,HAAA合并HLH更为罕见,常隐匿起病且病情严重。本文报告一例HAAA合并HLH病例,并总结文献中既往报道的病例,为该病的早期诊断和治疗提供参考。

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[Analysis of five cases of hepatitis associated aplastic anemia presenting with hemophagocytic lymphohistiocytosis at onset].
Zhonghua Er Ke Za Zhi. 2020 Mar 2;58(3):213-217. doi: 10.3760/cma.j.issn.0578-1310.2020.03.010.

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