[Research Advances in Pathogenesis of Idiopathic Multicentric Castleman Disease].

Yu-Han Gao, Jian Li, Lu Zhang
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Abstract

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening lymphoproliferative 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 underscores 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.

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[特发性多中心 Castleman 病发病机制的研究进展]。
特发性多中心性卡斯特曼病(iMCD)是一种罕见的、危及生命的淋巴组织增生性疾病,涉及全身炎症症状、多克隆淋巴细胞增生、全血细胞减少和多器官功能障碍。虽然白细胞介素-6介导的细胞因子风暴被认为是 iMCD 的主要驱动因素,但超过 50% 的患者对针对白细胞介素-6 或其受体的治疗无效。幸运的是,近年来有关 iMCD 发病机制的研究取得了显著进展。本文综述了该领域的最新进展,旨在为进一步研究提供理论基础,并促进治疗策略的优化,以改善患者的预后。
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来源期刊
中国医学科学院学报
中国医学科学院学报 Medicine-Medicine (all)
CiteScore
0.60
自引率
0.00%
发文量
6813
期刊介绍: Acta Academiae Medicinae Sinicae was founded in February 1979. It is a comprehensive medical academic journal published in China and abroad, supervised by the Ministry of Health of the People's Republic of China and sponsored by the Chinese Academy of Medical Sciences and Peking Union Medical College. The journal mainly reports the latest research results, work progress and dynamics in the fields of basic medicine, clinical medicine, pharmacy, preventive medicine, biomedicine, medical teaching and research, aiming to promote the exchange of medical information and improve the academic level of medicine. At present, the journal has been included in 10 famous foreign retrieval systems and their databases [Medline (PubMed online version), Elsevier, EMBASE, CA, WPRIM, ExtraMED, IC, JST, UPD and EBSCO-ASP]; and has been included in important domestic retrieval systems and databases [China Science Citation Database (Documentation and Information Center of the Chinese Academy of Sciences), China Core Journals Overview (Peking University Library), China Science and Technology Paper Statistical Source Database (China Science and Technology Core Journals) (China Institute of Scientific and Technological Information), China Science and Technology Journal Paper and Citation Database (China Institute of Scientific and Technological Information)].
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