Journal of Nippon Medical School
Online ISSN : 1347-3409
Print ISSN : 1345-4676
ISSN-L : 1345-4676
Case Reports
Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis with Central Nervous System Symptoms
Masahiro Sakaguchi, Yasunobu Nagata, Yasuhiro Terasaki, Atsushi Takeyoshi, Shunichi Yasuda, Shunsuke Honma, Ryosuke Kinoshita, Atsushi Marumo, Toshio Asayama, Shunsuke Yui, Satoshi Wakita, Muneo Okamoto, Yusuke Kajimoto, Koichi Inokuchi, Hiroki Yamaguchi
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Keywords: EBV-HLH, central nervous system infiltration
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2023 Volume 90 Issue 1 Pages 126-135

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) involves pathological histiocytes and phagocytosis of normal blood cells through activation of inflammatory cytokines. We report a case of Epstein-Barr virus-HLH in a 75-year-old woman who presented with fever, thrombocytopenia, and loss of consciousness. Epstein-Barr virus-HLH was diagnosed after we identified massive hemophagocytosis in bone marrow and Epstein-Barr virus DNA in cerebrospinal fluid. The HLH-2004 protocol was applied, and lactate dehydrogenase levels-which reflect HLH disease status-decreased. However, persistent loss of consciousness and multiple organ failure led to the patient's death on day 18. Most cases of primary and secondary HLH involve pediatric patients; adult cases are rare. Few cases of central nervous system involvement in older adults have been reported. Therefore, accumulation of more data will help in developing better treatment strategies.

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