特发性多中心型Castleman病的分型和治疗研究进展  

Research progress in the classification and treatment of idiopathic multicentric Castleman disease

作　　者：张路[1] 李剑[1] ZHANG Lu;LI Jian(Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing,100730,China)

机构地区：[1]中国医学科学院北京协和医院血液内科,北京100730

出　　处：《临床血液学杂志》2025年第1期31-33,50,共4页Journal of Clinical Hematology

基　　金：中国医学科学院临床与转化医学研究专项(No:2023-I2M-C&T-B-045)。

摘　　要：特发性多中心型Castleman病(idiopathic multicentric Castleman disease,iMCD)是一种罕见血液疾病。2017年,国际Castleman病协作组(Castleman disease collaborative network,CDCN)在Blood杂志上发表了国际首个iMCD诊断共识,首次将该病分为iMCD-NOS和iMCD-TAFRO两个亚型,奠定了该病诊断和分型的基础。2018年,CDCN同样在Blood杂志上发表了全球首个iMCD治疗指南,奠定了白细胞介素-6靶向治疗在该病中的重要地位。前述共识/指南发布以来,通过国内外研究者的持续努力,对该病的分型和治疗(尤其是非白细胞介素-6为靶点的治疗)进行了新的探索。文章旨在回顾和介绍iMCD分型和治疗领域的研究进展。Idiopathic multicentric Castleman disease(iMCD)is a rare hematological disease.In 2017,Castleman disease collaborative network(CDCN)published the first international consensus on diagnostic criteria for iMCD which further classified this disease into iMCD-NOS and iMCD-TAFRO.In 2018,CDCN published the first international evidence-based treatment guidelines for iMCD which emphasized the role of interleukin-6(IL-6)targeted therapy.Since publication of these two excellent works in Blood,researchers worldwide have been contributing in the classification and treatment(especially therapy directed against targets other than IL-6-signaling pathway)of this rare disease.This review aims to give a brief delineation of progress in the classification and treatment of iMCD since 2018.

关 键 词：特发性多中心型Castleman病 分型 治疗 研究进展 

分 类 号：R551.2[医药卫生—血液循环系统疾病]