ARTICOLI ORIGINALI   

Minerva Pediatrica 2015 June;67(3):251-61

Copyright © 2015 EDIZIONI MINERVA MEDICA

lingua: Inglese

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

Xue H., Chen C., Li W., Lin C., Fang J., Li Y., Xu H. ✉

Department of Pediatrics, Affiliated Sun Yat‑sen Memorial Hospital of Zhongshan University, Guangzhou, Guangdong Province, China

AIM: This study aims to analyze the prognostic risk factors in children with Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH).
METHODS: Seventy-four EBV-HLH patients were divided into two groups according to the specificity: clinical remission after four-week 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 ≤4 cm, and patients with WBC≥3×109/L had longer survival time than those with WBC<3×109/L, which exhibited significant differences.
CONCLUSION: Age negatively influences the early remission of EBV-HLH. WBC<3×109/L and spleen >4 cm exhibited high correlation with the prognosis of EBV-HLH.