32例儿童噬血细胞综合征临床特点及预后分析

Clinical Characteristics and Prognosis of 32 Children with Hemophagocytic Syndrome

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;感染相关性HLH以EBV感染最多见;死亡组与存活组血小板、总蛋白、白蛋白、胆碱酯酶、K^(+)、Ca^(2+)和脾肿大有统计学差异(P <0.05);多因素Logistic回归分析白蛋白低是患儿死亡的独立危险因素(P <0.05)。结论儿童HLH临床表现复杂,病死率高,一旦诊断清楚,需及时加强支持治疗和化疗,白蛋白是影响HLH患儿预后的独立危险因素。

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.