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

Abstract

Hemophagocytic lymphohistiocytosis (HLH) involves pathological histiocytes and phagocytosis of nor-mal 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 de-hydrogenase 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 devel-oping better treatment strategies. (J Nippon Med Sch 2023; 90: 126―134).