中国实用儿科杂志2012，Vol.27Issue(7)：513-516,4.
儿童EB病毒感染相关性噬血细胞淋巴组织细胞增生症死亡病例分析
Death analysis of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children
摘要
Abstract
Objective To explore the main causes of deceased cases and factors related with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH) fatality. Methods A retrospective study was performed on EBV-HLH cases admitted to Beijing Children's Hospital between June 2003 and October 2010. All patients' medical records were reviewed and analyzed. Follow-up was taken to get the prognosis information of all cases. Statistical evaluation was conducted using multivariate and univariate analysis. Results In the 90 cases of follow-up results, 32 were alive while another 58 were deceased and die overall fatality rate was 64.4%. Most patients (33/36) without undergoing chemotherapy died in short time after diagnosis of HLH (usually less than two months). The fatality rate was as high as 87.8% in non-chemotherapy group while it was 44.9% in chemotherapy group. Despite being given chemotherapy, 8 patients died within 2 months of diagnosis while 14 cases died 2 months after diagnosis. Seven of 8 patients suffered from severe infection, while 6 cases had severe coagulopathy and 7 cases had multiple organ failure. Causes of late deaths mainly included non-remission of the disease after chemotherapy (9 in 14) and relapse of HLH (4 in 14). The fibrino-gen was lower in deceased group than in survived group, while lactate dehydrogenase was higher. Logistic regression analysis showed that longer than 4 weeks of illness prior to diagnosis (OR=3.436) , non-chemotherapy (OR=11.09) and lower fibrino-gen level (OR= 1.866) were independent factors related with EBV-HLH fatality. Conclusion EBV-HLH is a severe disease in pediatric patients with poor outcome and high fatality rate. Severe infection, coagulopathy and multiple organ failure are the main reasons which cause the early deaths, while non-remission of the disease after chemotherapy or relapse of HLH cause deaths after two months of diagnosis. Longer than 4 weeks of illness prior to diagnosis, non-chemotherapy and lower fibrinogen level are factors related to EBV-HLH fatality.关键词
Epstein-Barr病毒/噬血细胞淋巴组织细胞增生症/死亡/危险因素Key words
Epstein-Barr virus/ hemophagocytic lymphohistiocytosis/ fatality/ risk factors分类
医药卫生引用本文复制引用
陈兰勤,刘春艳,谢正德,申昆玲..儿童EB病毒感染相关性噬血细胞淋巴组织细胞增生症死亡病例分析[J].中国实用儿科杂志,2012,27(7):513-516,4.基金项目
北京市自然科学基金资助项目(7113152) （7113152）
