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

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≥3×10(9)/L had longer survival time than those with WBC<3×10(9)/L, which exhibited significant differences.

CONCLUSION

Age negatively influences the early remission of EBV-HLH. WBC<3×10(9)/L and spleen>4 cm exhibited high correlation with the prognosis of EBV-HLH.