50例成人继发性噬血细胞综合征临床特点及预后分析

Analysis on clinical features and prognosis of 50 adult secondary hemophagocytic lymphohitiocytosis

目的:分析成人继发性噬血细胞综合征(HLH)的临床特点及预后。方法:回顾性分析我院50例成人继发性HLH患者的临床特点,分析发病原因、生存时间以及预后特点等。结果:淋巴瘤为引起成人继发性HLH的最主要诱因(46%),其次为感染(30%),有18%的患者未找到明确病因;确诊后1个月以内为生存率下降最快时间;肿瘤、感染以及未找到病因患者的生存率无明显差别;EB病毒感染患者总生存率低于未感染者,使用依托泊苷与未使用依托泊苷的患者生存率无明显差别。结论:淋巴瘤为继发性HLH最常见的诱因,EB病毒感染患者的存活率低,预后差,依托泊苷的使用需全面结合患者病情。

Objective：To analyze the clinical features and prognosis of adult secondary hemophagocytic lymphohitiocytosis（HLH）.Method：We retrospectively analyzed clinical characteristics of 50 cases of adult secondary HLH,and analyzed the cause of disease,treatment,survival time and prognosis etc.Result：Lymphoma was the primary cause of adult secondary HLH（46%）,followed by infection（30%）,and the pathogens of 18% patients were unclear.Survival rate decreased rapidly in the first month after diagnosis.There was no significant difference in survival rates among patients with tumor,infection,or disease with unclear etiology.The overall survival rate of patients with EB virus infection was lower than that of uninfected patients.There was no significant differencein of survival rate between the treatment with and without etoposide.Conclusion：Lymphoma is the most common cause of secondary HLH,survival rate of EB virus infected patients is low,and the comprehensive use of etoposide should depend on the patients＇ condition.