噬血细胞综合征所致肝功能异常1例并文献复习

Liver dysfunction caused by hemophagosytic syndrome:a case report and literature review

噬血细胞综合征是一个罕见的、危及生命的疾病,其主要特征为免疫系统过度刺激所致的系统性炎症、细胞因子风暴及多器官衰竭。根据促发因素的不同,噬血细胞综合征被分为原发性和继发性两类。噬血细胞综合征的治疗包括病因治疗及诱导缓解治疗,但治疗效果差。本文报道了1例继发性噬血细胞综合征导致的肝功能异常的诊治过程,并复习噬血细胞综合征所致的临床表现、诊断、治疗及预后以及噬血细胞综合征所致的肝功能异常的特点。

Hemophagocytic syndrome is a rare and life-threatening disease characterized by systemic inflammation,cytokine storm and multiple organ dysfunction caused by overstimulation of the immune system.According to precipitating factor,hemophagocytic syndrome are classified as“primary”and“secondary”.The treatment of hemophagocytic syndrome includes etiological treatment and induction of remission,however,the therapeutic effect is poor.This article reported the diagnosis and treatment of a case of liver dysfunction caused by secondary hemophagocytic syndrome,and reviewed the clinical manifestations,diagnosis,treatment and prognosis of hemophagocytic syndrome and the characteristics of liver dysfunction caused by hemophagocytic syndrome.