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

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• 作者：Shino Magaki ; MD ; PhD^a ; ^{smagaki@mednet.ucla.edu} ; Nora Ostrzega ; MD^b ; Elliot Ho ; DO^c ; Catherine Yim ; MD^d ; Phillis Wu ; MD^c ; Harry V. Vinters ; MD^a ; ^e ; ^f
• 关键词：HLH ; hemophagocytic lymphohistiocytosis ; EBV ; Epstein-Barr virus ; CNS ; central nervous system
• 刊名：Human Pathology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：59
• 期：Complete
• 页码：108-112
• 全文大小：1181 K
• 卷排序：59

文摘

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.