Abstract: Objective To evaluate the clinical features of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis(EBV-HLH) in children and to explore its prognosis and risk factors. Methods A retrospective study was performed on 78 pediatric patients with EBV-HLH who were admitted to Beijing Children's Hospital between 2003 and 2008. All patients' medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory and outcome information was collected. Statistical evaluation was conducted using multivariate and univariate analysis. Results The age of onset peaked between 1 and 2 years. EBV-HLH occurred mainly in the patients who were positive for IgG antibody to EBV nuclear antigen (70.5%). The overall fatality of the disease was 56.7%. Twelve of the 39 case(30.8%) died rapidly within 2 months after diagnosis. Multivariate analysis revealed that not receiving chemotherapy(P=0.002), ≥4 weeks of illness prior to diagnosis(P=0.004), and albumin levels <20 g/L(P=0.045) significantly predicted an increased risk of death. Conclusion EBV-HLH is a severe disease with a high fatality rate that occurs mainly after EBV reactivation. Early initiation of chemotherapy and timely diagnosis significantly improve survival rate. Practical strategies should focus on reducing the likelihood of early death.

Key words: Epstein-Barr virus, hemophagocytic lymphohistiocytosis, risk factor