Analysis of prognostic risk factors in children with Epstein-Barr virus-associated hemophagocytic syndrome

作者：Xue, H; Chen, C; Li, W^*; Lin, C; Fang, J; Li, Y; Xu, H

来源：Minerva Pediatrica, 2015, 67(3): 251-261.

• Child
• Lymphohistiocytosis, hemophagocytic
• Epstein-Barr virus infections
• Prognosis

Aim. This study aims to analyze the prognostic risk factors in children with Epstein-Barr virus-associated haemophagocytic lympho-histiocytosis (EBV-HLH).
Methods. Seventy-four EBV-HLH patients were divided into two groups according to the specificity: clinical remission after fourweek inductive therapy group and active disease group; death group and survival group. The risk factors that affect early efficacy and prognosis were analyzed.
Results. Overall survival rate of the 74 children was 75.7%, while the recurrence rate was 13.5%. The one-year survival rate was 71.4 +/- 5.6%, and the three-year survival rate was 65.9 +/- 6.6%, with a median survival rate of 40 +/- 19.9 months. The multivariate logistic regression analysis showed that age was the primary risk factor that affected the first 4 weeks alleviation, and the severity of splenomegaly and WBC level upon hospitalization were the risk factors that affected the prognosis. Patients with spleen >4 cm had shorter survival time than those with spleen cm, and patients with WBC >= 3x10(9)/L had longer survival time than those with WBC<3x10(9)/L, which exhibited significant differences.
Conclusion. Age negatively influences the early remission of EBV-HLH. WBC<3x10(9)/L and spleen >4 cm exhibited high correlation with the prognosis of EBV-HLH.

• 出版日期2015-6
• 单位中山大学