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

Abstract

Objective: To analyze the clinical characteristics, prognosis factors and risk factors of chronic active Epstein-Barr virus infection in children.

Study design: Observational analysis of baseline data and follow-up evaluation data of children with chronic active Epstein-Barr virus infection in our center between January 1, 2016 and December 31, 2019; they were followed through June 30, 2020.

Results: There were 96 children with chronic active Epstein-Barr virus infection (50 male and 46 female children), with the median age of 6.7 years (range from 0.6 to 17.6 years) at diagnosis. The median follow-up time was 16.5 months. The 3 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). Epstein-Barr virus infected only T lymphocytes, natural killer cells, or both T- and natural killer-cell types in 15 (33.3%), 17 (37.8%), and 13 (28.9%), respectively. At the end of follow-up, 26 children had died, 60 survived, and 10 were lost to follow-up. Generally, progression-free survival was 69.8% ± 2.4%. The level of interleukin (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 .018, respectively).

Conclusions: Children 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.

Keywords: children; chronic active Epstein-Barr virus; clinical characteristics; prognostic factors.