論文

査読有り 国際誌
2011年8月4日

Rapid diagnosis of FHL3 by flow cytometric detection of intraplatelet Munc13-4 protein.

Blood
  • Yuuki Murata
  • Takahiro Yasumi
  • Ryutaro Shirakawa
  • Kazushi Izawa
  • Hidemasa Sakai
  • Junya Abe
  • Naoko Tanaka
  • Tomoki Kawai
  • Koichi Oshima
  • Megumu Saito
  • Ryuta Nishikomori
  • Osamu Ohara
  • Eiichi Ishii
  • Tatsutoshi Nakahata
  • Hisanori Horiuchi
  • Toshio Heike
  • 全て表示

118
5
開始ページ
1225
終了ページ
30
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1182/blood-2011-01-329540
出版者・発行元
AMER SOC HEMATOLOGY

Familial hemophagocytic lymphohistiocytosis (FHL) is a potentially lethal genetic disorder of immune dysregulation that requires prompt and accurate diagnosis to initiate life-saving immunosuppressive therapy and to prepare for hematopoietic stem cell transplantation. In the present study, 85 patients with hemophagocytic lymphohistiocytosis were screened for FHL3 by Western blotting using platelets and by natural killer cell lysosomal exocytosis assay. Six of these patients were diagnosed with FHL3. In the acute disease phase requiring platelet transfusion, it was difficult to diagnose FHL3 by Western blot analysis or by lysosomal exocytosis assay. In contrast, the newly established flow cytometric analysis of intraplatelet Munc13-4 protein expression revealed bimodal populations of normal and Munc13-4-deficient platelets. These findings indicate that flow cytometric detection of intraplatelet Munc13-4 protein is a sensitive and reliable method to rapidly screen for FHL3 with a very small amount of whole blood, even in the acute phase of the disease.

リンク情報
DOI
https://doi.org/10.1182/blood-2011-01-329540
J-GLOBAL
https://jglobal.jst.go.jp/detail?JGLOBAL_ID=201202257821662026
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/21653941
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000293510000013&DestApp=WOS_CPL
ID情報
  • DOI : 10.1182/blood-2011-01-329540
  • ISSN : 0006-4971
  • J-Global ID : 201202257821662026
  • PubMed ID : 21653941
  • Web of Science ID : WOS:000293510000013

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