目的　探讨EB 病毒相关噬血细胞综合征的临床与病理特征。方法　回顾性分析1 例EB 病毒相关噬血细胞综合征并重症肝炎死亡病例的临床资料，复习相关文献。结果　患儿1 岁2 个月，有发热、血细胞减少、纤维蛋白原、自然杀伤细胞下降、血清铁蛋白及三酰甘油升高；血涂片异常淋巴细胞占5%；EBV-DNA 荧光定量检测1.39×108 拷贝/ml；尸检结果提示死亡原因为多脏器功能衰竭；肝脏组织行EB 病毒编码RNAs(EBERs) 原位杂交检测，约30% 淋巴细胞中EBERs 阳性，肝细胞EBERs 阴性。结论　该例患儿确认EB 病毒相关噬血细胞综合征，累及多器官，其中肝损伤并非EB 病毒直接感染所致。

Objective　To understand the clinical and pathological features of Epstein-Barr virus (EBV) associated hemophagocytic syndrome.Methods　The clinical data from one deceased pediatric patient of EBV associated hemophagocytic syndrome combined with severe hepatitis were retrospectively analyzed. The relevant literatures were reviewed. Results　The 1 year and 2 months old patient presented with fever, cytopenia, decreased fibrinogen and natural killer cells, elevated serum ferritin and triglycerides. The abnormal lymphocytes accounted for 5% in blood smear. There were 1.39×108 EBV-DNA copies/ml by fluorescence quantitative detection The autopsy results suggest that the cause of death was multiple organ failure. The EB virus encoded RNAs (EBERs) were detected in liver tissues by in situ hybridization. Approximately 30% lymphocytes were EBERs positive and the hepatocytes were EBERs negative. Conclusions　The patient was confirmed to have EBV associated hemophagocytic syndrome involving multiple organs. However the liver damage was not caused by the direct infection of EB virus.