EB病毒感染相关的成人噬血细胞综合征临床分析

Clinical analysis for adult patients with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis

目的:探讨EB病毒(EBV)感染相关的成人噬血细胞综合征(EBV-HLH)患者的临床特征。方法:对12例EBV-HLH患者的临床资料进行回顾性分析。结果:所有患者均以发热、血细胞减少为主要表现,多数患者伴有肝、脾肿大及不同程度的肝功损害和凝血异常,骨髓内可见到吞噬自身血细胞的组织细胞。7例患者为单纯EBV感染,另外5例患者为淋巴瘤并发EBV感染。治疗基于HLH-2004方案或CHOP-E化疗,最终有11例患者死亡,死亡原因为出血3例,感染性休克2例,肝衰及多脏器功能衰竭6例。结论:成人EBV-HLH临床表现凶险,对免疫化疗的疗效不佳;使用依托泊苷治疗可延长患者生存时间。

Objective：To investigate the clinical characteristics for adult patients with EBV-associated hemophagocytic lymphohistiocytosis（EBV-HLH）.Method：The clinical features of 12 patients with EBV-HLH were retrospectively analyzed.Result：All the patients had fever as well as hypocytomsis,mostly with hepatomegaly,splenomegalia and various degrees of hepatic lesion and dysfunction of coagulation.Hemophagocytes were found in bone marrow.Seven patients were triggered only by Epstein-Barr virus infection and other five patients were complicated with lymphoma.Therapeutic regimens were based on HLH-2004 protocol or CHOP-E regimen.By far,eleven of twelve patients died of various causes,including hemorrhage（3 cases）,infectious shock（2 cases） as well as liver failure and multiple organ failure（6 cases）.Conclusion：Adult patients with EBV-HLH had a very poor prognosis.Most of them were refractory to immunochemotherapy.Treatment with etoposide could prolong their life.