Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis with Central Nervous System Symptoms
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- Sakaguchi Masahiro
- Department of Hematology, Nippon Medical School
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- Nagata Yasunobu
- Department of Hematology, Nippon Medical School
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- Terasaki Yasuhiro
- Department of Analytic Human Pathology, Nippon Medical School Division of Pathology, Nippon Medical School Hospital
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- Takeyoshi Atsushi
- Department of Hematology, Nippon Medical School
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- Yasuda Shunichi
- Department of Hematology, Nippon Medical School
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- Honma Shunsuke
- Department of Hematology, Nippon Medical School
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- Kinoshita Ryosuke
- Department of Hematology, Nippon Medical School
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- Marumo Atsushi
- Department of Hematology, Nippon Medical School
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- Asayama Toshio
- Department of Hematology, Nippon Medical School
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- Yui Shunsuke
- Department of Hematology, Nippon Medical School
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- Wakita Satoshi
- Department of Hematology, Nippon Medical School
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- Okamoto Muneo
- Department of Hematology, Nippon Medical School
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- Kajimoto Yusuke
- Department of Analytic Human Pathology, Nippon Medical School
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- Inokuchi Koichi
- Department of Hematology, Nippon Medical School
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- Yamaguchi Hiroki
- Department of Hematology, Nippon Medical School
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説明
<p>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.</p>
収録刊行物
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- 日医大誌
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日医大誌 90 (1), 126-135, 2023-02-25
日本医科大学医学会