# Fatal refractory chronic active Epstein-Barr virus infection with hemophagocytic lymphohistiocytosis and NK/T-cell lymphoma: a case report

## TL;DR

A 28-year-old woman died from a rare and severe Epstein-Barr virus complication involving liver damage and lymphoma, highlighting the need for early diagnosis and treatment.

## Contribution

This case report highlights the rare and fatal progression of chronic active EBV infection with HLH and NK/T-cell lymphoma.

## Key findings

- The patient had EBV-positive T-cells in liver tissue, consistent with chronic active EBV infection and HLH.
- Despite aggressive treatment, the patient succumbed to the disease, underscoring its severity.
- Early recognition and expert consultation are critical for managing this rare condition.

## Abstract

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.

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.

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.