ABSTRACT Background Epstein-Barr virus (EBV) is most often associated with asymptomatic infection or infectious mononucleosis syndrome. A rare and fatal underestimated complication is chronic active EBV infection (CAEBV) with hemophagocytic lymphohistiocytosis (HLH) and B or NK/T-cell lymphoma. Case Summary A 28-year-old woman presented with fevers, malaise, fatigue, abdominal pain, nausea, vomiting, and episodic skin rashes. Laboratory tests revealed pancytopenia, significantly elevated liver enzymes, and serology consistent with primary EBV infection, with very high EBV DNA in serum. All other causes of hepatitis were excluded. Liver biopsy showed EBV-positive T-cells infiltrating the liver parenchyma with erythrophagocytosis, consistent with CAEBV infection with HLH. Despite aggressive interventions, the patient ultimately succumbed to grave complications and refractory disease. Conclusion CAEBV is a rare and fatal complication of EBV. Clinician education, early recognition, and expert consultation improve outcomes. Treatment so far consists of chemotherapy followed by hematopoietic stem cell transplantation.
Mielke et al. (Mon,) studied this question.