噬血性淋巴组织细胞增生症,简称噬血细胞综合征(HPS),是一组较为少见、由活化的淋巴细胞和组织细胞增生引起的多器官严重炎症反应而又免疫无效的临床综合征,起病急、病情进展迅速、病死率高。主要特征为发热、肝脾大、全血细胞减少和组织细胞噬血现象。感染是引起HPS的最常见触发因素,EB病毒(EBV)感染在HPS发病 Hemophagocytic lymphohistiocytosis, referred to as hemophagocytic syndrome (HPS), is a group of more rare clinical syndromes that are immunologically ineffective due to activation of multiple organ inflammation caused by lymphocytic and histiocytic hyperplasia Urgent, rapid progression, high mortality. The main features of fever, hepatosplenomegaly, pancytopenia and tissue hemophagocytosis. Infection is the most common trigger for HPS and Epstein-Barr virus (EBV) infection occurs in HPS