A case of acute encephalopathy with hemophagocytic lymphohistiocytosis and clonal T-cell expansion
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概要
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We report on a 9-year-old boy who presented with acute encephalopathy and hemophagocytic lymphohistiocytosis (HLH). The patient was referred to our hospital because of fever, seizures, and decreased consciousness. He showed moderately elevated levels of proinflammatory cytokines in the cerebrospinal fluid and plasma, and clonal expansion of highly activated CD8 + T cells in the peripheral blood. These CD8 + T cells were found to be larger cells that stained positive for T-cell receptor Vβ13.6, and decreased shortly after steroid therapy. Our findings suggest that his acute encephalopathy was likely a clinical manifestation of HLH, and that immunophenotypic analysis may be helpful for early recognition of HLH in such rare encephalopathy. © 2011 The Japanese Society of Child Neurology.
- 2012-05-01
著者
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Niida Yo
Department Of Pediatrics Faculty Of Medicine Kanazawa University
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Asai Erika
Department Of Pediatrics School Of Medicine Institute Of Medical Pharmaceutical And Health Sciences
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Kuroda Mondo
Department Of Pediatrics Graduate School Of Medical Science Faculty Of Medicine Kanazawa University
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Toma Tomoko
Department Of Angiogenesis And Vascular Development School Of Medicine Kanazawa University
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Wada Taizo
Department Of Angiogenesis And Vascular Development School Of Medicine Kanazawa University
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Yachie Akihiro
Department Of Angiogenesis And Vascular Development School Of Medicine Kanazawa University
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VAN VU
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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Nishimura Kashiku
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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Vu Quang
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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TOMA TOMOKO
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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YACHIE Akihiro
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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WADA Taizo
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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KURODA Mondo
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
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