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

Research paper by Shino S Magaki, Nora N Ostrzega, Elliot E Ho, Catherine C Yim, Phillis P Wu, Harry V HV Vinters

Indexed on: 31 Aug '16Published on: 31 Aug '16Published in: Human Pathology



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 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.

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