Severe chronic active Epstein-Barr virus infection associated with multiple necrotic lesions in the liver

We report an atypical presentation of a chronic active Epstein-Barr virus (EBV) infection with multiple nodular coagulation necrosis in the liver, that appeared as hypodense areas on a CT scan. The patient, a 26-year-old man, was hospitalized following over 2 years of intermittent fever, weight loss and liver abnormalities after contracting infectious mononucleosis. We diagnosed his illness as a chronic active EBV infection (CAEBV) because of the high antibody titers against EBV and the histological evidence of organ disease with demonstration of EBV mRNA. A liver biopsy revealed EBV-infected T-cell infiltration with hemophagocytosis and marked hepatocytolytic necrosis. The patient developed multiple coagulation necrosis with well-defined borders surrounding T-cell aggregation in the liver 8 months later. He died of respiratory failure due to interstitial pneumonitis. The analysis of EBV-genome termini demonstrated a clonal proliferation of T-cells harboring EBV, but no T-cell antigen receptor (TCR) gene rearrangement was observed. We speculate that the pathogenesis of this disease was an atypical expression of organ damage as a result of an aberrant T-cell response to EBV infection.