EBV病毒感染相关噬血细胞综合征的诊治分析  

The diagnosis and treatment of EBV infection related to hemophagocytic syndrome

作　　者：何秋连[1] 曹红[1] 马春蓉[1] 全凤琼[1] 贾晋松[2] 

机构地区：[1]川北医学院第二临床医学院.南充市中心医院血液内科,四川南充637000 [2]北京大学人民医院血液科,北京100083

出　　处：《西部医学》2017年第5期689-693,共5页Medical Journal of West China

摘　　要：目的探讨EBV病毒感染相关噬血细胞综合征患者的发病机制及临床诊治。方法对1例EBV病毒感染相关噬血细胞综合征(HLH)患者依据诊断指南进行诊断,使用甲强龙160mg q12h/天联合人免疫球蛋白20g/天共3天抑制免疫风暴治疗,同时降血氨、抗感染、纠正肝功及输注新鲜冰冻血浆、人血白蛋白、纤维蛋白原及血小板等支持治疗,对病例资料进行总结分析。结果患者经治疗后体温明显下降,生命体征稳定,病情有好转过程,但该患者最终因肝性脑病、呼吸功能衰竭而死亡。结论通过对本例HLH患者使用大剂量甲强龙及丙球冲击治疗阻断细胞因子风暴,同时联合控制感染、输注新鲜冰冻血浆、纤维蛋白原等支持治疗,能控制免疫失衡,并取得了积极的治疗效果。故大剂量丙种球蛋白联合大剂量甲强龙方案适用于多器官功能障碍的极重症HLH患者。Objective To explore the mechanism and clinical diagnosis and treatment of EBV virus associated hemophagocytic syndrome. Methods 1 patients was diagnosed with diagnostic guidelines. The patient was treated with methylprednisolone and immunoglobulin. At the same time, the patient was treated with lowering blood ammonia, anti-infection, correcting liver function and supportive treatment. The clinical data were analyzed. Results After treatment, the temperature of the patient used decrease and vital signs used to be stable, but the patient died with hepatic encephalopathy and respiratory failure. Conclusion Large dose of gamma globulin in combination with large dose methylprednisolone scheme is more suitable for patients with extremely severe HLH of multiple organ dysfunction.

关 键 词：嗜血细胞综合征 EBV病毒感染 抑制免疫风暴治疗 多器官功能障碍 

分 类 号：R552[医药卫生—血液循环系统疾病]