Research Advances in Pathogenesis of Idiopathic Multicentric Castleman Disease
10.3881/j.issn.1000-503X.16008
- VernacularTitle:特发性多中心型Castleman病发病机制的研究进展
- Author:
Yuhan GAO
1
;
Jian LI
;
Lu ZHANG
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院血液科,北京 100730
- Keywords:
idiopathic multicentric Castleman disease;
pathogenesis;
treatment
- From:
Acta Academiae Medicinae Sinicae
2024;46(5):756-762
- CountryChina
- Language:Chinese
-
Abstract:
Idiopathic multicentric Castleman disease(iMCD)is a rare and life-threatening lymphopro-liferative disorder involving systemic inflammatory symptoms,polyclonal lymphoproliferation,cytopenia,and multiple organ dysfunction.Although interleukin-6-mediated cytokine storm is thought to be the key driver of iMCD,more than 50％of the patients fail to respond to the treatments targeting interleukin-6 or its receptors.This under-scores the urgent need to identify other cytokines and pathogenic pathways that may underlie iMCD.Fortunately,recent years have witnessed notable research advances in the pathogenesis of iMCD.This article reviews the latest progress in this field,aiming to provide a theoretical foundation for further research and facilitate the optimization of treatment strategies to improve the prognosis of the patients.
