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  • Title: Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus in the central nervous system.
    Author: Magaki S, Ostrzega N, Ho E, Yim C, Wu P, Vinters HV.
    Journal: Hum Pathol; 2017 Jan; 59():108-112. PubMed ID: 27574808.
    Abstract:
    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 are essential because 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 is rare and not well characterized neuropathologically. We report a case of fatal EBV-associated HLH with severe involvement of the central nervous system showing florid hemophagocytosis in the choroid plexus, with extensive neuron loss and gliosis in the cerebrum, cerebellum, and brainstem.
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