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目的探讨影响EB病毒相关性噬血淋巴组织细胞增生症(EBV-HLH)死亡危险因素,以指导EBV-HLH患儿的治疗,降低病死率。方法 对1999~2008年在本院收治的29例EBV-HLH患儿,根据预后分为生存组和死亡组。对两组患儿性别、年龄、临床表现、实验室检查、治疗情况进行单因素分析和多因素非条件Logistic回归分析。结果 29例中,死亡11例,病死率为37.93%。单因素分析结果显示,影响EBV-HLH死亡的因素包括年龄、肺部感染、血清铁蛋白(SF)、乳酸脱氢酶(LDH)、骨髓增生程度、C-反应蛋白(CRP)、采用噬血细胞综合征-2004(HLH-04)方案。多因素非条件Logistic回归分析,肺部感染、血清铁蛋白、年龄、采用HLH-04方案有统计学意义,其中采用HLH-04方案是死亡的保护因素,而年龄、肺部感染、血清铁蛋白升高是死亡的危险因素。结论 有肺部感染、SF水平越高、年龄越小、未采用HLH-04方案的EBV-HLH患儿死亡的危险度较高,因此,在临床上,应考虑以上因素,确诊后尽快采用HLH-04方案治疗,以提高患儿的生存率。
Objective To investigate the risk factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in order to guide the treatment of EBV-HLH children and reduce the mortality. Methods 29 children with EBV-HLH admitted to our hospital from 1999 to 2008 were divided into survival group and death group according to the prognosis. Two groups of children with gender, age, clinical manifestations, laboratory tests, treatment univariate analysis and multivariate non-conditional Logistic regression analysis. Results Among the 29 cases, 11 died and the case fatality rate was 37.93%. Univariate analysis showed that the factors affecting the mortality of EBV-HLH included age, pulmonary infection, serum ferritin (SF), lactate dehydrogenase (LDH), myeloproliferation, C-reactive protein (CRP) Syndrome-2004 (HLH-04) program. Multivariate non-conditional Logistic regression analysis, pulmonary infection, serum ferritin, age, using HLH-04 regimen was statistically significant, of which HLH-04 regimen was the protective factor of death, while age, pulmonary infection, serum ferritin Elevation is a risk factor for death. Conclusions In patients with lung infection, the higher the SF level and the younger the risk of death from EBV-HLH patients who did not adopt HLH-04 regimen was higher. Therefore, in clinical practice, the above factors should be taken into account. HLH -04 program treatment to improve the survival rate of children.