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Differential diagnosis of Japanese patients with CLL and related disorders

Research Project

Project/Area Number 16K08963
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Laboratory medicine
Research InstitutionNiigata University

Principal Investigator

TAKIZAWA JUN  新潟大学, 医歯学系, 准教授 (70463990)

Co-Investigator(Kenkyū-buntansha) 曽根 博仁  新潟大学, 医歯学系, 教授 (30312846)
青木 定夫  新潟薬科大学, 薬学部, 教授 (40242408)
鈴木 律朗  島根大学, 学術研究院医学・看護学系, 准教授 (20280810)
大島 孝一  久留米大学, 医学部, 教授 (50203766)
Research Collaborator Suzumiya Junji  
Project Period (FY) 2016-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2018: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2017: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2016: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
KeywordsCLL / CLLRSG-01 / LEF1 / Matutes' score / Typical CLL / Atypical CLL / IGHV / CLL-like LPD / Matutes score / FISH / MYD88 / 診断学 / 造血器腫瘍 / 遺伝子
Outline of Final Research Achievements

Chronic lymphocytic leukemia (CLL), the most frequent adult leukemia in Western countries, is rare in Japan. To clarify the characteristics of Japanese patients with CLL, we conducted a nationwide registry study (CLLRSG-01). The WHO classification was used to establish a central diagnosis in all cases. Analyses of flow cytometry, FISH, and the mutational status of IGHV were performed. Morphologically 162 cases were classified as CLL and 59 as other B-cell neoplasms. Immunophenotypically, 109 of the 162 cases were confirmed as CLL (classic CLL), but the other 53 patients could not be attributed to a diagnosis of CLL (CLL-like LPD). The frequency of morphologically atypical CLL in the CLL-like LPD group was higher than that in the classic CLL group. Our analyses indicate that the proportion of Japanese patients with classic CLL is much lower among those with small mature B-cell neoplasms.

Academic Significance and Societal Importance of the Research Achievements

慢性リンパ性白血病(CLL)は本邦において希少疾患であり、欧米と形態評価の方法が異なっていたため(欧米は自然乾燥なのに対して本邦は強制乾燥を用いる)、正確な実態は明らかにされていなかった。今回、欧米と同じ手法を用いて、国内多施設共同研究を行うことにより、初めて本邦CLLの実態が多数例で評価された。典型的形態と免疫形質を有するCLLの頻度が低いことが初めて明らかになり、非典型例が多数存在することが示された。今後、典型例と非典型例の分子病態を比較することにより、本邦に特徴的な発症機序が明らかになれば新規治療に結びつく可能性が高く、学術的意義や社会的意義は大きいものと考えられる。

Report

(4 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • 2016 Research-status Report
  • Research Products

    (8 results)

All 2018 2017 2016 Other

All Int'l Joint Research (1 results) Journal Article (3 results) (of which Peer Reviewed: 2 results,  Open Access: 2 results) Presentation (4 results) (of which Int'l Joint Research: 1 results,  Invited: 1 results)

  • [Int'l Joint Research] Medical University of Vienna(Austria)

    • Related Report
      2016 Research-status Report
  • [Journal Article] Identification of the BRAF V600E mutation in Japanese patients with hairy cell leukemia and related diseases using a quenching probe method2018

    • Author(s)
      Itamura Hidekazu、Ide Masaru、Sato Akemi、Sueoka-Aragane Naoko、Sueoka Eisaburo、Nishida Aya、Masunari Taro、Aoki Sadao、Takizawa Jun、Suzumiya Junji、Kimura Shinya
    • Journal Title

      International Journal of Hematology

      Volume: 108 Issue: 4 Pages: 416-422

    • DOI

      10.1007/s12185-018-2506-3

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] 慢性リンパ性白血病(CLL)と類縁疾患の鑑別診断2018

    • Author(s)
      瀧澤 淳、桐生真依子、河本啓介
    • Journal Title

      血液フロンティア

      Volume: 28 Pages: 179-185

    • Related Report
      2017 Research-status Report
  • [Journal Article] Chronic lymphocytic leukemia: pathophysiology and current therapy2017

    • Author(s)
      瀧澤 淳
    • Journal Title

      Rinsho Ketsueki

      Volume: 58 Issue: 5 Pages: 471-479

    • DOI

      10.11406/rinketsu.58.471

    • NAID

      130005690180

    • ISSN
      0485-1439, 1882-0824
    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] Characteristics of chronic lymphocytic leukemia in Japan (CLLRSG-01 study)2017

    • Author(s)
      Takizawa J, Suzuki R, Kiguchi T, et al.
    • Organizer
      International Workshop on Chronic Lymphocytic Leukemia (XVII iwCLL 2017)
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] シンポジウム⑤ 「リンパ腫の治療」 本邦におけるCLLの治療~現状と今後の展望~2017

    • Author(s)
      瀧澤 淳
    • Organizer
      第57回日本リンパ網内系学会総会
    • Related Report
      2017 Research-status Report
    • Invited
  • [Presentation] Characteristics of small mature B-cell LPD resembling CLL in the Japanese population (CLLRSG-01)2017

    • Author(s)
      Takizawa J, Suzuki R, Izutsu K, et al.
    • Organizer
      第79回日本血液学会学術集会
    • Related Report
      2017 Research-status Report
  • [Presentation] CD13 positive chronic lymphocytic leukemia -A distinct subset of CLL-.2016

    • Author(s)
      内山孝由、青木定夫、瀧澤淳、中村直哉、宇都宮與、伊豆津宏二、麻奥英毅、木口亨、大島孝一、鈴木律朗、鈴宮淳司.
    • Organizer
      第76回日本血液学会総会
    • Place of Presentation
      パシフィコ横浜(神奈川県横浜市)
    • Year and Date
      2016-10-13
    • Related Report
      2016 Research-status Report

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Published: 2016-04-21   Modified: 2020-03-30  

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