A case of acute encephalopathy with hemophagocytic lymphohistiocytosis and clonal T-cell expansion

• Wada, Taizo

• Nishimura, Kashiku

• Kuroda, Mondo

• Asai, Erika

• Vu, Quang Van

• Toma, Tomoko

• Niida, Yo

• Yachie, Akihiro

抄録

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.

収録刊行物

• Brain and Development

  Brain and Development 34 (5), 376-379, 2012-05-01

  Elsevier / 日本小児神経学会Japanese Society of Child Neurology

キーワード

• Acute encephalopathy
• CD8 +
• Clonal T-cell expansion
• Hemophagocytic lymphohistiocytosis

詳細情報

• CRID: 1050845760922428160
• NII論文ID: 10031050761
• NII書誌ID: AA00111153
• ISSN: 03877604
• Web Site: http://hdl.handle.net/2297/31375
• Web Site: https://search.jamas.or.jp/link/ui/2013156086
• 本文言語コード: en
• 資料種別: journal article
• データソース種別:
  • IRDB
  • CiNii Articles