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An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection
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Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus in the central nervous system

Shino Magaki1, Nora Ostrzega2, Elliot Ho3

  • 1Section of Neuropathology, Department of Pathology and Laboratory Medicine, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732, USA.

Human Pathology
|August 31, 2016
PubMed

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.

Keywords:
AutopsyCentral nervous systemEpstein-Barr virusHemophagocytic lymphohistiocytosisImmunohistochemistry

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