Language

English

Publication Date

3-1-2025

Journal

ACG Case Reports Journal

DOI

10.14309/crj.0000000000001649

PMID

40115891

PMCID

PMC11925415

PubMedCentral® Posted Date

3-20-2025

PubMedCentral® Full Text Version

Post-print

Abstract

Systemic chronic active Epstein-Barr virus (EBV) disease is a rare, delayed, and life-threatening complication of EBV infection. It is characterized by persistent viremia and a mononucleosis-like syndrome with multisystemic involvement because of lymphocytic tissue infiltration. We present the case of an 82-year-old woman without known immunodeficiency who presented with abnormal liver enzymes and interstitial pneumonia. Investigations revealed hyperferritinemia, elevated soluble interleukin-2 receptor levels, hypofibrinogenemia, and hepatosplenomegaly, consistent with hemophagocytic lymphohistiocytosis, as well as a high EBV viral load. Liver biopsy demonstrated portal chronic lymphocytic inflammation and positive EBV in situ hybridization, suggesting systemic chronic active Epstein-Barr virus disease as the underlying etiology.

Keywords

sCAEBV, EBV, HLH, Hepatitis, interstitial pneumonia

Published Open-Access

yes

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