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(hemato-poietic 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.
Abstract
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
Key words
chronic active EBV infection/lymphoproliferative disease/pathogenesis/diagnosis/treatment
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