伴EB病毒血症的噬血细胞性淋巴组织细胞增生症80例临床分析

Clinical analyses of 80 hemophagocytic lymphohistiocytosis patients with Epstein-Barr viremia

目的探讨伴EB病毒血症的噬血细胞性淋巴组织细胞增生症（HLH）的临床特征、治疗及预后情况。方法对2008至2013年在北京友谊医院确诊为HLH且初次外周血EB病毒DNA检测中〉1000拷贝／ml的80例患者（年龄≥14岁）的临床资料进行回顾性分析。结果80例伴EB病毒血症的HLH患者中最终确诊为EB病毒相关HLH46例，淋巴瘤相关HLH30例，原发性HLH4例。EB病毒相关HLH与淋巴瘤相关HLH两组间的实验室指标只有丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AST）的差异有统计学意义（P=0．021、0．035）。中位随访时间2．0个月（0．5～20．0个月）。80例患者总体1、3、6、12个月的生存率分别为58．8％、37．5％、29．7％、19．6％。结论伴有EB病毒血症的HLH患者病情凶险，预后差，常规实验室指标对于区分EB病毒相关HLH与淋巴瘤相关的HLH的意义不大。

Objective To analyze the clinical characteristics, treatment and prognosis of hemophagocytic lymphohistiocytosis （HLH） patients with Epstein-Barr viremia （EBV）. Methods A retrospective study was conducted to analyze the clinical data of 80 HLH patients aged t≥14 years with EBV-DNA 〉 1 000 copies/ml in peripheral blood from 2008 to 2013. Results There were EBV-HLH （ n = 46）, HLH-associated lymphoma （n = 30） and primary HLH （n = 4 ）. Among the relevant laboratory parameters, inter-group statistical differences existed only in alanine transaminase （ALT） and aspartate aminotransferase （AST） （P = 0. 021,0. 035）. The median follow-up period was 2. 0 （0. 5 -20. 0） months. And the 1-month, 3-month, 6-month and 12-month overall survival rates were 58. 8% , 37. 5%, 29.7% and 19. 6% respectively. Conclusions HLH patients with EBV have a pernicious clinical course with a poor prognosis. And it makes little sense for distinguishing HLH-associated lymphoma from EBV-HLH through routine laboratory tests.