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Establishing the research basis for innovative medical care of familial hemophagocytic lymphohistiocytosis.

Research Project

Project/Area Number 25670475
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Pediatrics
Research InstitutionKyoto University

Principal Investigator

HEIKE Toshio  京都大学, 医学(系)研究科(研究院), 教授 (90190173)

Co-Investigator(Kenkyū-buntansha) YASUMI Takahiro  京都大学, 大学院医学研究科発達小児科学, 講師 (00511891)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2013: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Keywords血球貪食性リンパ組織球症 / 免疫調節異常症 / 機能解析 / 創薬基盤開発 / FHL / 血球貪食症候群 / NK細胞 / 細胞障害性T細胞
Outline of Final Research Achievements

We established a system to provide comprehensive screening of FHL to all HLH patients by the detection of perforin expression in NK cells; Munc13-4, syntaxin-11, and Munc18-2 expression in platelets; by NK cell and CTL degranulation assays; and by genetic analysis. We compared the levels of serum sIL2R, ferritin, and other readily available laboratory parameters among pediatric HLH patients during the early stage of the disease, and showed that these parameters can be used to predict the underlying etiology and to efficiently identify patients who need molecular screening for FHL.(Yasumi, et al. British J Haematology, 2015)
We also established a system to analyze the cytolytic activity and the degranulation capacity of CTL lines derived from HLH patients. In addition, we have succeeded in evaluating the effect of a UNC13D mutation by transfecting the mutated cDNA construct into CTL lines established from a FHL3 patient.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • Research Products

    (5 results)

All 2015 2014

All Journal Article (2 results) (of which Peer Reviewed: 1 results,  Acknowledgement Compliant: 1 results) Presentation (3 results) (of which Int'l Joint Research: 1 results,  Invited: 1 results)

  • [Journal Article] Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis2015

    • Author(s)
      Yasumi T, Hori M, Hiejima E, Shibata H, Izawa K, Oda H, Yoshioka K, Nakagawa K, Kawai T, Nishikomori R, Ohara O, Heike T
    • Journal Title

      British Journal of Haematology

      Volume: 170 Issue: 4 Pages: 532-538

    • DOI

      10.1111/bjh.13461

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Heterogeneity of HLH pathophysiology and treatment strategies2015

    • Author(s)
      八角高裕、柴田洋史、下寺佐栄子、平家俊男
    • Journal Title

      Rinsho Ketsueki

      Volume: 56 Issue: 10 Pages: 2248-2257

    • DOI

      10.11406/rinketsu.56.2248

    • NAID

      130005103313

    • ISSN
      0485-1439, 1882-0824
    • Related Report
      2015 Annual Research Report
  • [Presentation] HLH病態の多様性と治療戦略の展望2015

    • Author(s)
      八角高裕
    • Organizer
      第77回日本血液学会
    • Place of Presentation
      ANAクラウンプラザホテル金沢
    • Year and Date
      2015-10-18
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] CD57+ CTL degranulation and Munc13-4 protein expression assays are sensitive and reliable screening methods for FHL32015

    • Author(s)
      Kikuya A, Hori M, Yasumi T, Hiejima E, Shibata H, Izawa K, Oda H, Nishikomori R, Ohara O, Heike T
    • Organizer
      第11回アジア小児研究学会
    • Place of Presentation
      大阪国際会議場
    • Year and Date
      2015-04-15
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 当科におけるFHL(家族性血球貪食性リンパ組織球症)スクリーニングの現状2014

    • Author(s)
      堀雅之
    • Organizer
      第117回日本小児科学会学術集会
    • Place of Presentation
      名古屋国際会議場
    • Year and Date
      2014-04-13
    • Related Report
      2014 Research-status Report

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Published: 2014-07-25   Modified: 2019-07-29  

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