噬血细胞综合征的临床诊治进展

Clinical advances of hemophagocytic syndrome

噬血细胞综合征（HPS）是一组致命性的免疫功能紊乱性疾病,分为原发性及继发性HPS两大类.该病病情复杂,临床表现缺乏特异性,确诊难度大,疾病进展迅速,病死率高,预后差.诊断主要依据HPS-2004方案诊断标准.治疗首选依托泊苷、地塞米松、环孢素.本文主要就HPS相关病因、诊治及预后相关进展进行综述.

Hemophagocytic syndrome is a group of fatal immune function disorder,which can be divided into primary and secondary HPS.The disease is complex,lacking of specificity,difficult in diagnosis with quick progress,high mortality and poor prognosis.The diagnosis is mainly based on the HPS-2004 diagnostic criteria,etoposide,dexamethasone and cyclosporine would be first chosen for treatment.This article reviews the clinical advances on etiology,diagnosis,treatment and prognosis of hemophagocytic syndrome.