儿童噬血细胞综合征预后危险因素分析

Analysis of prognostic risk factors in childhood hemophagocytive syndrome

目的探讨儿童噬血细胞综合征（HPS）预后相关危险因素。方法采用回顾性分析的方法，对2007年3月至2011年3月我院收治的50例HPS患儿血清学、病理学改变及预后资料进行系统分析。按随访患儿的生存情况分为生存组和死亡组，采用单因素和多因素Logistic回归分析影响患儿预后的危险因素。结果50例HPS患儿中男30例，女20例，发病年龄3个月～10岁，46例患儿血清白蛋白、NK细胞及胆碱酯酶较正常值范围降低，大部分患儿血清铁蛋白升高伴有凝血功能异常和脂质代谢紊乱。40例患儿骨髓中出现吞噬细胞，35例患儿EBV—IgM抗体阳性，随访37例患儿中25例死亡，其中13例于住院1个月内死亡。单因素分析结果显示，与生存组比较死亡组患儿血清白蛋白、胆碱酯酶、NK细胞活性均降低，凝血酶原时间延长。多因素Logistic回归分析结果显示，病程〉1个月、白蛋白〈25g／L、胆碱酯酶〈2000U／L，NK细胞活性为0—3％、EBV—IgM抗体阳性与预后显著相关（P值均〈0．05）。结论HPS患儿病情凶险，病死率高，病程〉1个月、白蛋白低、胆碱酯酶和NK细胞活性低、EBV—IgM抗体阳性是影响患儿预后的主要危险因素。

Objective To identify and explore the prognostic risk factors of the hemophagocytive syndrome（HPS）. Methods A retrospective study was conducted on 50 childhood patients with HPS who were admitted to our hospital between 2007 and 2011. All their medical records were reviewed and analyzed. For each patient, demographic, laboratory data and outcome information were collected. The patients were divided into deceased or survived groups based on the follow-up results. Comparative analysis of the data was done by using independent-samples test and logistic multiple and univariate regression. Results Among the 50 HPS patients, 30 were male and 20 female, age ranged from 3 months to 10 years. Reduction of serum albumin, cholinesterase and natural killer（NK） cells was found in the forty-six patients. The laboratory features showed an elevation of serum fcrritin with hypofibrinogenemia and hypertriglyceridemia in most of the patients. Forty of patients had hemophagocyte in bone marrow at dignosis of HPS. The positive serum EBV-IgM was found in thirty-five patients. During the observation period, 25 of 37 patients （ 67.6% ） died, while 13 of whom died within a month after hospitalization. The deceased patients were more likely to have lower albumin, cholines- terase, NK cells level and more prolonged active partial thromboplastin time than the survived patiens （P 〈 0.05 ）. Multivariate logistic regression analysis revealed that duration of illness 〉 1 month, albumin level 〈 25 g/L, cholinesterase level 〈 2000 U/L, NK cell level 0 -3% and positive EBV-IgM were related with the prognosis significantly（ P 〈 0.05 for all comparisons）. Conclusion This study revealed that duration of illess 〉 1 month, decreases in albumin, NK cell and cholinesterase, and positive EBV-IgM were the risk factors related to mortality in children.