摘要
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。
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.
出处
《临床血液学杂志》
CAS
2018年第5期729-732,共4页
Journal of Clinical Hematology
基金
国家自然科学基金(No:81570175)
