Epstein-Barr病毒相关噬血细胞综合征（EBV-HLH）病情进展快，病死率高。高细胞因子血症是其发病重要机制，临床表现多样。诊断需符合HLH诊断标准并存在EBV感染的证据。治疗首选依托泊苷、糖皮质激素、环孢素A联合应用。如化疗效果不佳，可行造血干细胞移植。现就该病的最新研究进展进行综述。

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a rapidly progressive disease with a high rate of mortality. In the patients with EBV-HLH, hypercytokinemia plays a major pathogenetic middle mechanism. The clinical manifestations were various and its diagnosis should meet HLH 2004 criteria as well as EBV-positive. In terms of treatment, the combination of dexamethasone, etoposide and cyclosporin A is the first choice. If they do not work，hematopoietic stem cell transplantation can be done. The recent progress on diagnosis and treatment of EBV-HLH is reviewed in the present paper.