Clinical Features and Prognostic Factors of Children With Chronic Active Epstein-Barr Virus Infection: A Retrospective Analysis of a Single-Center.

Abstract

OBJECTIVE\nTo analyze the clinical characteristics, prognosis factors and risk factors of chronic active Epstein-Barr virus (EBV) infection in children.\n\n\nSTUDY DESIGN\nObservational analysis of baseline data and follow-up evaluation data of children with chronic active EBV infection in our center between January 1, 2016 and December 31, 2019, and were followed through June 30, 2020.\n\n\nRESULTS\nThere were 96 children with chronic active EBV infection, including 50 males and 46 females, with the median age of 6.7 years (range from 0.6-17.6 years) at diagnosis. The median follow-up time was 16.5 months. The three most common clinical manifestations were fever, lymph node enlargement, and hepatomegaly or splenomegaly. Thirty-three patients (36.3%) also had a diagnosis of hemophagocytic lymphohistiocytosis (HLH). EBV infected only T lymphocytes, NK cells, or both T- and NK-cell types in 15 (33.3%), 17 (37.8%), and 13 (28.9%), respectively. At the end of follow up, 26 children had died and 60 survived, 10 were lost to follow up. Generally, progression-free survival was 69.8% ± 2.4%. The level of IL-6 and IL-10 and the combination of younger age and lower pathologic grade at diagnosis were independent prognostic factors by Cox regression analysis (P = .009 and 0.018, respectively).\n\n\nCONCLUSIONS\nChildren with lower levels of IL-6 and IL-10, or with younger age and lower pathologic grades, generally had favorable outcomes at the terminal point of follow up, indicating better prognostic signs.