摘要
<正>噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH)又称噬血细胞综合征,是一种罕见的致命性免疫调节异常综合征,以过度的免疫反应及过度活化的巨噬细胞和T淋巴细胞广泛浸润各组织器官,导致多器官系统功能衰竭为特征~([1])。HLH包括原发性和继发性两种类型。其中继发性HLH主要由感染、肿瘤、风湿免
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis(EBV-HLH)is one of the most common forms of the secondary HLH.Patients diagnosed as having EBV-HLH was based on fulfilling the HLH-2004 criteria and the diagnosis of EBV infection.HLH-2004 protocol is still the first choice of the treatment of EBV-HLH.In recent years,as the research continues on how to treat EBV-HLH,more and more individual and stratified therapeutic strategies have been chosen.In this review,the research progress on the epidemiology,pathogenesis,diagnosis,therapy and prognosis of EBV-HLH have been summarized.
引文
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