特发性多中心型Castleman病的诊疗进展

Castleman病(Castleman disease,CD)为一种罕见的淋巴增生性疾病,临床上分为单中心型Castleman病(unicentric Castleman disease,UCD)和多中心型Castleman病(multicentric Castleman disease,MCD)。根据人类疱疹病毒-8(human herpes virus-8,HHV-8)感染状态,将MCD进一步分为HHV-8阳性和HHV-8阴性,后者也称特发性多中心型Castleman病(idiopathic multicentric Castleman disease,iMCD)。UCD和HHV-8阳性的MCD均已有标准的治疗方案,而iMCD的诊疗缺乏统一标准,主要凭借临床医生的经验。近年来,国际上新定义了iMCD的概念与诊断标准,并于2018年底公布了iMCD的治疗共识。本文旨在介绍iMCD的治疗现状和最新进展,以期对临床医生诊治Castleman病给予参考和借鉴。

Advance in diagnosis and treatment of idiopathic multicentric Castleman disease

Castleman disease (CD) is a rare lymphoproliferative disease that is clinically classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). According to the status of human herpes virus-8 (HHV-8) infection, MCD is further classified into HHV-8-positive MCD, and HHV-8-negative MCD, which is also called idiopathic multicentric Castleman disease (iMCD). There are standard treatment options for both UCD and HHV-8-positive MCD, but there has been no uniform standard of diagnosis and treatment for iMCD, which mainly relied on the experience of clinicians. In recent years, Castleman Disease Collaborative Network (CDCN) has newly defined the concept and diagnostic criteria of iMCD, and a comprehensive guidance on the treatment of iMCD was established in November 2018. In this paper, we try to review the current treatment options and advances of iMCD, which might help Chinese clinicians to diagnose and treat this rare disease.