Investigation of biomarker in idiopathic multicentric Castleman disease
Project/Area Number |
20K07407
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Review Section |
Basic Section 49020:Human pathology-related
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Research Institution | Okayama University |
Principal Investigator |
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Project Period (FY) |
2020-04-01 – 2023-03-31
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Project Status |
Completed (Fiscal Year 2022)
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Budget Amount *help |
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2022: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2021: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2020: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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Keywords | 特発性多中心性Castleman病 / IPL / TAFRO / NOS / 分類 / iMCD / RNA-seq / Castleman disease / キャッスルマン病 / 新規バイオマーカー |
Outline of Research at the Start |
HHV8陰性・形質細胞型キャッスルマン病(以下PC-type iMCD)と膠原病に遺伝子レベルで境界線があるか否か、そしてその指標となる分子病理学的バイオマーカーを明らかにすることである。PC-type iMCDと病理診断されたにもかかわらず、後に関節リウマチやSLEの診断基準を満たす例を経験することもある。そこで本研究では、PC-type iMCDは均一な疾患単位になるか否かを、高感度ゲノミクス手法を用いて、分子学的および病理学的に検証し、その得られたデータを元に明確な診断指標になり得る新規分子病理学的バイオマーカーを見出し、病態解明に繋げたい。
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Outline of Final Research Achievements |
iMCD is classified into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) and iMCD-NOS (not otherwise specified). The former has been established as a relatively homogeneous disease unit that has been recently re-defined, while the latter is considered to be a heterogeneous disease that could be further divided into several subtypes. In 1980, Mori et al. proposed the concept of idiopathic plasmacytic lymphadenopathy (IPL), a disease presenting with polyclonal hypergammaglobulinemia and a sheet-like proliferation of mature plasma cells in the lymph nodes. In our study indicated that IPL is a clinicopathologically uniform entity that forms an independent subtype of iMCD.
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Academic Significance and Societal Importance of the Research Achievements |
これまでiMCDは臨床的にiMCD-NOSとiMCD-TAFROの2群に分類されていた。しかし我々の研究において、IPLは病態形成メカニズムも均一な疾患であることが示され、この結果を踏まえ、2022年12月に開催された国際コンセンサス会議において、これまで2群に分類されていたiMCDにiMCD-IPLが新たに加わり、3群に分類されることとなった。すなわちこれまでiMCD-NOSに含まれていたIPLが世界に認められる疾患単位となった。
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Report
(4 results)
Research Products
(26 results)
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[Journal Article] Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions2023
Author(s)
Nakayama Yuki、Mizuno Hiroki、Sawa Naoki、Suwabe Tatsuya、Yamanouchi Masayuki、Ikuma Daisuke、Hasegawa Eiko、Hoshino Junichi、Sekine Akinari、Oba Yuki、Kono Kei、Kinowaki Keiichi、Ohashi Kenichi、Suzuki Kodai、Sato Yasuharu、Shimizu Akira、Yamaguchi Yutaka、Ubara Yoshifumi
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Journal Title
Internal Medicine
Volume: 62
Issue: 15
Pages: 2223-2229
DOI
ISSN
0918-2918, 1349-7235
Year and Date
2023-08-01
Related Report
Peer Reviewed / Open Access
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[Journal Article] Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?2022
Author(s)
Nishimura Y, Nishikori A, Sawada H, Czech T, Otsuka Y, Nishimura MF, Mizuno H, Sawa N, Momose S, Ohsawa K, Otsuka F, Sato Y.
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Journal Title
Journal of Clinical and Experimental Hematopathology
Volume: 62
Issue: 2
Pages: 99-105
DOI
ISSN
1346-4280, 1880-9952
Related Report
Peer Reviewed / Open Access
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