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A Journal on Pediatrics, Neonatology, Adolescent Medicine,
Child and Adolescent Psychiatry

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

language: English

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.

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