慢性活动性EB病毒感染的发病机制和诊治进展  

Pathogenesis,diagnosis and treatment of chronic active Epstein-Barr virus infection

作　　者：艾军红 王然 谢正德 AI JunHong;WANG Ran;XIE Zheng(De Key Laboratory of Major Diseases in Children,Ministry of Education,National Clinical Research Center for Respiratory Diseases,Research Unit of Critical Infection in Children,Chinese Academy of Medical Sciences,2019RU016,Laboratory of Infection and Virology,Beijing Pediatric Research Institute,Beijing Children’s Hospital,Capital Medical University,National Center for Children’s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院,北京市儿科研究所感染与病毒研究室,中国医学科学院儿童危重感染诊治创新单元,国家呼吸系统疾病临床医学研究中心,儿科重大疾病研究教育部重点实验室,北京100045

出　　处：《中国科学：生命科学》2024年第12期2321-2329,共9页Scientia Sinica(Vitae)

基　　金：北京市自然科学基金(批准号:L242117);中国医学科学院医学与健康科技创新工程(创新单元)(批准号:2019-I2M-5-026);首都卫生发展科研专项(批准号:2024-4-1142)资助。

摘　　要：慢性活动性EBV感染(chronic active Epstein-Barr virus infection,CAEBV)是EBV感染T和NK细胞所致的一种淋巴增殖性疾病.其主要临床表现为传染性单核细胞增多症样症状反复发生或持续数月至数年,伴肝脾肿大,外周血EBV-DNA载量升高,可发生噬血细胞性淋巴组织细胞增生症、白血病、淋巴瘤等严重并发症,预后差,病死率高.由于其发病机制尚未完全阐明,国内外尚无统一的治疗方案.目前,化疗联合造血干细胞移植(hematopoietic stem cell transplantation,HSCT)是唯一有效的根治方法.本文综述了CAEBV发病机制、诊断及治疗等方面取得的研究进展,并对治疗中存在的问题进行了总结,旨在为CAEBV的临床诊断和治疗提供参考.Chronic active Epstein-Barr virus infection(CAEBV)is a lymphoproliferative disease caused by EBV infection in T cells and NK cells.Patients mainly present with symptoms resembling infectious mononucleosis,occurring repeatedly or persisting for months to years,accompanied by hepatosplenomegaly,elevated peripheral blood EBV DNA levels,and severe complications such as hemophagocytic lymphohistiocytosis,leukemia,and lymphoma.The prognosis is poor,with a high mortality rate.Due to the incomplete understanding of its pathogenesis,there is currently no unified treatment protocol internationally.Presently,chemotherapy combined with hematopoietic stem cell transplantation is considered the only effective curative method.This article reviews the research progress in the pathogenesis,diagnosis,and treatment of CAEBV,highlighting the existing challenges in its management.

关 键 词：慢性活动性EBV感染 淋巴增殖性疾病 发病机制 诊断 治疗 

分 类 号：R511[医药卫生—内科学]