Investigation of biomarker in idiopathic multicentric Castleman disease

2022 Fiscal Year Final Research Report

• Project/Area Number: 20K07407
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 49020:Human pathology-related
• Research Institution: Okayama University
• Principal Investigator: Sato Yasuharu 岡山大学, 保健学域, 教授 (00579831)
• Project Period (FY): 2020-04-01 – 2023-03-31
• 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.

Free Research Field

病理学

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。