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

Autor: Masahiro, Sakaguchi, Yasunobu, Nagata, Yasuhiro, Terasaki, Atsushi, Takeyoshi, Syunichi, Yasuda, Shunsuke, Honma, Ryosuke, Kinoshita, Atsushi, Marumo, Toshio, Asayama, Shunsuke, Yui, Satoshi, Wakita, Muneo, Okamoto, Yusuke, Kajimoto, Koiti, Inokuchi, Hiroki, Yamaguchi
Rok vydání: 2023
Předmět:
Zdroj: Journal of Nippon Medical School. 90:126-135
ISSN: 1347-3409
1345-4676
Popis: 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.
Databáze: OpenAIRE