摘要慢性活动性EB病毒感染(chronic active Epstein-Barr virus infection.CAEBV)表现为持续存在或反复出现的传染性单核细胞增多症样(infectious Mononucleosis,IM)的临床症状,以持续或间歇发热、淋巴结病、肝脾肿大及肝功能损害为主要临床特点且不存在明确诊断的潜在疾病.CAEBV具体的发病机制尚不完全明确,患者外周血中EBV感染的T细胞、NK细胞或B细胞异常克隆增殖,伴有异常增高的EBV载量.患者可伴发噬血细胞综合征、多器官衰竭、恶性淋巴瘤等预后差甚至威胁生命的疾病.目前异基因干细胞移植是唯一有效的治疗手段.本文就国内外CAEBV临床及基础研究方面的进展进行综述.

abstractsChronic active EBV (CAEBV) infection presents with persistent or recurring IM-like symptoms including fever,hepatosplenomegaly,lymphadenopathy,and liver dysfunction without definite underlying diseases at the diagnosis.No causative pathogenetic mechanisms have been sufficiently clarified,these patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells,NK cells and B cells.Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis,diverse organ failure,malignant lymphomas and so on.Allogeneic hematopoietic stem cell transplantation (Allo-HSCT) is the only effective therapy at present.This review outlines the recent progress of CAEBV clinical and fundamental research at home and abroad.