Epstein–Barr virus–related hemophagocytic lymphohistiocytosis with central nervous system symptoms

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.