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

J Nippon Med Sch. 2023 Mar 11;90(1):126-135. doi: 10.1272/jnms.JNMS.2023_90-105. Epub 2022 Nov 25.

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.

Keywords: EBV-HLH; central nervous system infiltration.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Central Nervous System
  • Child
  • Epstein-Barr Virus Infections* / complications
  • Female
  • Herpesvirus 4, Human
  • Humans
  • Lymphohistiocytosis, Hemophagocytic* / complications
  • Unconsciousness / complications