Hemophagocytic lymphohistiocytosis associated with Epstein–Barr virus in the central nervous system

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune hyperactivation syndrome which may be primary (genetic) or secondary to various immune-related conditions including infection, immunodeficiency, and malignancies. Rapid diagnosis and treatment is essential as it can be associated with significant morbidity and mortality. Epstein–Barr virus (EBV) is a known infectious cause of acquired HLH, but EBV-associated HLH involving the central nervous system (CNS) is rare and not well characterized neuropathologically. We report a case of fatal EBV-associated HLH with severe involvement of the CNS showing florid hemophagocytosis in the choroid plexus, with extensive neuron loss and gliosis in the cerebrum, cerebellum, and brainstem.