-Case Reports-

Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis with Central Nervous System Symptoms

Masahiro Sakaguchi¹, Yasunobu Nagata¹, Yasuhiro Terasaki^2,3, Atsushi Takeyoshi¹, Shunichi Yasuda¹, Shunsuke Honma¹, Ryosuke Kinoshita¹, Atsushi Marumo¹, Toshio Asayama¹, Shunsuke Yui¹, Satoshi Wakita¹, Muneo Okamoto¹, Yusuke Kajimoto², Koichi Inokuchi¹ and Hiroki Yamaguchi¹

¹Department of Hematology, Nippon Medical School, Tokyo, Japan
²Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan
³Division of Pathology, Nippon Medical School Hospital, Tokyo, Japan

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.

J Nippon Med Sch 2023; 90: 126-134

Keywords
EBV-HLH, central nervous system infiltration

Correspondence to
Yasunobu Nagata, MD, PhD, Department of Hematology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
y-nagata@nms.ac.jp

Received, March 8, 2022
Accepted, June 17, 2022