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Clinical and subclinical features, and treatment outcomes of children with chronic active epstein-barr virus infection: A retrospective single-center analysis
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Abstract
Introduction: Chronic active Epstein-Barr virus (CAEBV) is a lymphoproliferative disorder associated with EBV infection. CAEBV is a rare but severe, potentially life-threatening illness. Clinical manifestations develop in multiple organ systems and mortality. This study aims to investigate clinical, subclinical features and treatment outcomes of children with CAEBV infection. Method: This retrospective study was conducted over 5 years, from January 2017 to December 2021, at the Vietnam National Children's Hospital (VNCH), the country's largest referral tertiary children's hospital. Children diagnosed with CAEBV during this period were included in the study. All statistical analyses were performed using SPSS software version 20.0. Results: There were 43 CAEBV patients, of which 21(48.8%) showed hemophagocytic syndrome (HLH–EBV) at the time of diagnosis. The median age of onset was 41 months, the male/female ratio was 2.3/1 and 21(48.8%) patients experienced severe outcomes or death during the study period. Symptoms include fever (100%), hepatomegaly (97.6%), splenomegaly (83.4%), and lymphadenopathy (46.5%). There are changes in hematologic, biochemical tests and immune cell counts in CAEBV. The high EBV viral load in serum remained stable after receiving antiviral treatment, with a median of 2.32 x 105 cp/ml. Conclusions: Although many measures, such as antivirals, immunosuppressants, chemicals and intravenous immunoglobulin are adopted, the disease has a high mortality rate. More research is needed to clarify clinical characteristics, the roles of diagnostic tests and treatment measures for this group of patients.
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Keywords
Children, HLH, EBV, CAEBV
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