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 signifi...

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Bibliographic Details
Published in:Human pathology 2017-01, Vol.59, p.108-112
Main Authors: Magaki, Shino, MD, PhD, Ostrzega, Nora, MD, Ho, Elliot, DO, Yim, Catherine, MD, Wu, Phillis, MD, Vinters, Harry V., MD
Format: Article
Language:English
Subjects:
Adult
Autopsy
Biopsy
Bone marrow
Brain
Brain - pathology
Brain - virology
Central nervous system
Central Nervous System Viral Diseases - diagnosis
Central Nervous System Viral Diseases - therapy
Central Nervous System Viral Diseases - virology
Cytokines
Cytotoxicity
Disease
Epstein-Barr virus
Epstein-Barr Virus Infections - diagnosis
Epstein-Barr Virus Infections - therapy
Epstein-Barr Virus Infections - virology
Fatal Outcome
Fever
Gangrene
Gliosis
Hemophagocytic lymphohistiocytosis
Herpesvirus 4, Human - genetics
Herpesvirus 4, Human - isolation & purification
Humans
Immunohistochemistry
Infections
Laboratories
Liver
Lymphocytes
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - therapy
Lymphohistiocytosis, Hemophagocytic - virology
Magnetic Resonance Imaging
Male
Medical imaging
Mortality
Mutation
Nervous system
Neurons - pathology
Neurons - virology
NMR
Nuclear magnetic resonance
Pathology
Patients
Pediatrics
RNA, Viral - genetics
Severity of Illness Index
Spinal Cord - pathology
Spinal Cord - virology
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Description
Summary: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.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2016.07.033