2022 Fiscal Year Final Research Report
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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Keywords | 特発性多中心性Castleman病 / IPL / TAFRO / NOS / 分類 |
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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Free Research Field |
病理学
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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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