title: Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus in the central nervous system.
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.
authors: Section 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. Electronic address: smagaki@mednet.ucla.edu.
authors: Shino
authors: S
authors: Magaki
authors: Department of Pathology, Olive View-University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA 91342, USA.
authors: Nora
authors: N
authors: Ostrzega
authors: Department of Internal Medicine, Olive View-University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA 91342, USA.
authors: Elliot
authors: E
authors: Ho
authors: Department of Radiology, Olive View-University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA 91342, USA.
authors: Catherine
authors: C
authors: Yim
authors: Department of Internal Medicine, Olive View-University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA 91342, USA.
authors: Phillis
authors: P
authors: Wu
authors: Section 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; Department of Neurology, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732, USA; Brain Research Institute, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732, USA.
authors: Harry V
authors: HV
authors: Vinters
mesh_terms: D000328:Adult; D001344:Autopsy; D001706:Biopsy; D001921:Brain / Q000473:pathology / Q000821:virology*; D020805:Central Nervous System Viral Diseases / Q000175:diagnosis / Q000628:therapy / Q000821:virology*; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000628:therapy / Q000821:virology*; D017809:Fatal Outcome; D005911:Gliosis; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007150:Immunohistochemistry; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000628:therapy / Q000821:virology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009474:Neurons / Q000473:pathology / Q000821:virology*; D012367:RNA, Viral / Q000235:genetics; D012720:Severity of Illness Index; D013116:Spinal Cord / Q000473:pathology / Q000821:virology*
chemical_list: D012367:RNA, Viral
keywords: Autopsy; Central nervous system; Epstein-Barr virus; Hemophagocytic lymphohistiocytosis; Immunohistochemistry