Fulminant liver failure due to Epstein-Barr virus in immunodeficiency disorders.

BACKGROUND

The majority of patients with Epstein-Barr virus (EBV)-associated hepatitis have a mild clinical course; most infections resolve spontaneously. However, in rare cases, or in patients with underlying immune deficits, outcomes can be fatal.

METHODS

We used a single-centre case series.

RESULTS

In this report, we describe two cases of acute EBV hepatitis. The first patient developed fulminant hepatitis with multi-organ failure and severe immune dysregulation; he died despite maximal intensive-care management. Post-mortem, his bone marrow biopsy results revealed an X-linked lymphoproliferative disease. This primary immunodeficiency impairs the body's ability to control EBV infection and is a risk factor for developing an EBV-positive T-cell lymphoma in childhood. The second patient presented with hepatitis, cytopenias, and hepatosplenomegaly in the context of persistent EBV viremia and responded well to treatment (dexamethasone, rituximab, and supportive care). Immunodeficiency testing was negative in this case.

CONCLUSIONS

Prompt multidisciplinary management is recommended in cases of severe EBV-associated liver injury.