目的 分析儿童HLH的临床特点，探讨其预后危险因素?方法 收集我科诊治32例HLH患儿临床症状?体征及实验室相关指标，并分为存活组(22例)与死亡组(10例)，统计学比较两组患儿指标的差异，分析与预后相关的危险因素?结果 32例HLH患儿，男14例?女18例，中位年龄2(1.02~9)岁，死亡10例?存活22例;临床表现为发热?肝脾肿大?黄疸?浆膜腔积液?水肿?皮疹和中枢神经系统病变等;实验室检查以血细胞减少?肝功能异常?凝血异常?高三酰甘油血症?NK降低?低蛋白血症?铁蛋白和sCD25升高为主;感染相关“,”Objective To analyze the clinical features and prognostic risk factors of children with HLH. Methods collecting clinical data of 32 children with HLH， dividing all the patients into survival group (n=22) and death group (n=10)， comparing the differences between the two groups. Results Among the 32 HLH patients，14 were male and 18 were female， the median age is 2(1.02~9) years old， 10 cases survive and 22 cases die. Clinical manifestations include fever，hepatosplenomegaly， jaundice， serous effusion， edema， rash and central nervous system lesions，etc. The main laboratory tests were hematocytopenia， abnormal liver function， abnormal coagulation， hypertriglyceridemia， decreased NK，hypoalbuminemia，elevated ferritin and scd25.Infection-related HLH is most common with EBV infection.There were significant differences in platelet， total protein， albumin， cholinesterase， K^+，Ca^2+ and splenomegalia between the death group and the survival group(P<0.05). Multivariate logistic regression analysis showed that low albumin was an independent risk factor (P <0.05).Conclusion Children with HLH have complex clinical manifestations and high mortality， and once the diagnosis is clear， intensive supportive treatment and chemotherapy are needed; Albumin is an independent risk factor for the prognosis of children with HLH.