论文部分内容阅读
目的探讨儿童EB病毒感染相关噬血细胞淋巴组织细胞增生症(Epstein-Barr virus associated hemophagocytic lymphohistiocytosis,EBV-HLH)的主要死因及相关危险因素。方法回顾性分析首都医科大学附属北京儿童医院2003年6月至2010年10月收治的103例EBV-HLH患儿临床资料,并对其转归进行随访。采用单因素及多因素分析方法进行统计学分析。结果本组失访13例。随访成功的90例患儿中,存活32例,死亡58例,病死率为64.4%。其中未进行化疗的36例患儿病死率高达87.8%,其中33例在诊断后2个月内死亡;接受化疗患儿(化疗组)的病死率为44.9%。化疗组8例诊断后早期死亡病例,其中7例发生重症感染;6例发生严重的凝血功能障碍;7例发生脏器功能衰竭。14例晚期死亡病例中,9例患儿在化疗后病情持续不缓解并最终死亡;4例在停药后出现复发而死亡。单因素分析显示死亡组较存活组患儿的纤维蛋白原水平更低,而乳酸脱氢酶更高(P1=0.033、P2=0.005,均<0.05);Logistics回归分析显示,发病-诊断时间大于4周、未进行化疗及低纤维蛋白原均为与EBV-HLH患儿死亡相关的危险因素,其死亡危险度分别为3.436、11.09和1.866。结论儿童EBV-HLH预后差、病死率高。重症感染、凝血功能障碍及脏器功能衰竭是早期死亡主要原因;持续疾病活动及复发为晚期死亡的主要原因。发病-诊断时间大于4周、未进行化疗及低纤维蛋白原水平为与EBV-HLH死亡相关的危险因素。
Objective To investigate the main causes of death and related risk factors of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children. Methods The clinical data of 103 children with EBV-HLH admitted to Beijing Children’s Hospital Affiliated to Capital Medical University from June 2003 to October 2010 were retrospectively analyzed. The prognosis was followed up. Univariate and multivariate analysis were used for statistical analysis. Results The group lost 13 cases. Of the 90 children who were followed up successfully, 32 survived and 58 died, with a case fatality rate of 64.4%. Among 36 cases without chemotherapy, the case fatality rate was 87.8%, of which 33 cases died within 2 months after diagnosis. The case-fatality rate of chemotherapy-treated children was 44.9%. In the chemotherapy group, 8 cases were diagnosed early deaths, of which 7 cases had severe infection, 6 cases had severe coagulation dysfunction and 7 cases had organ failure. Among the 14 cases of late death, 9 cases did not relieve and eventually died after chemotherapy. Four cases died of recurrence after stopping treatment. Univariate analysis showed that fibrinogen level was lower and lactate dehydrogenase was higher in death group than those in survival group (P <0.05). Logistic regression analysis showed that onset-diagnosis time was longer than 4 weeks, no chemotherapy and low fibrinogen are risk factors associated with death of EBV-HLH children, the risk of death were 3.436,11.09 and 1.866 respectively. Conclusion Children with EBV-HLH have poor prognosis and high mortality. Severe infection, coagulation disorders and organ failure are the main causes of early death; sustained disease activity and recurrence are the main causes of late death. Incidence-The diagnosis was longer than 4 weeks, with no chemotherapy and low fibrinogen levels as risk factors for death from EBV-HLH.