■Castleman病(CD)是一组罕见且异质性的淋巴增生性疾病,包括单中心CD(UCD),人类疱疹病毒-8(HHV-8)相关多中心CD(HHV8-MCD),和HHV-8阴性/特发性多中心CD(iMCD)。CD的知识主要来自病例系列或回顾性研究,但这些研究的纳入标准各不相同,因为Castleman病协作网络(CDCN)的iMCD和UCD诊断标准分别要到2017年和2020年才能使用.Further,这些标准和指南尚未得到系统评估。
■在这个国家,多中心,实施CDCN标准的回顾性研究,我们招募了1634例CD患者(UCD,n=903;MCD,n=731)从2000年到2021年在40个中国机构描述临床特征,治疗方案,和CD的预后因素。
■在UCD中,有162例(17.9%)患者出现MCD样炎症状态.在MCD中,有12例HHV8-MCD患者和719例HHV-8阴性MCD患者,其中包括139个无症状MCD(aMCD)和580个符合临床标准的iMCD.580例iMCD患者中,41(7.1%)符合iMCD-TAFRO标准,其他的是iMCD-NOS。iMCD-NOS进一步分为iMCD-IPL(n=97)和不含IPL的iMCD-NOS(n=442)。在具有一线治疗数据的iMCD患者中,观察到脉冲联合化疗向连续治疗的趋势。生存分析显示,亚型和重度iMCD之间存在显着差异(HR=3.747;95%CI:2.112-6.649,p<0.001)的预后较差。
■这项研究描绘了CD,中国的治疗选择和生存信息,并验证了CDCN对严重iMCD的定义与不良结局之间的关联,需要更密集的治疗。
■北京市科学技术委员会,CAMS创新基金和国家高级医院临床研究资助。
UNASSIGNED: Castleman disease (CD) is a group of rare and heterogenous lymphoproliferative disorders including unicentric CD (UCD), human herpesvirus-8(HHV-8)-associated multicentric CD (HHV8-MCD), and HHV-8-negative/idiopathic multicentric CD (iMCD). Knowledge of CD mainly comes from case series or retrospective studies, but the inclusion criteria of these studies vary because the Castleman Disease Collaborative Network (CDCN) diagnostic criteria for iMCD and UCD were not available until 2017 and 2020, respectively. Further, these criteria and guidelines have not been systematically evaluated.
UNASSIGNED: In this national, multicenter, retrospective study implementing CDCN criteria, we enrolled 1634 CD patients (UCD, n = 903; MCD, n = 731) from 2000 to 2021 at 40 Chinese institutions to depict clinical features, treatment options, and prognostic factors of CD.
UNASSIGNED: Among UCD, there were 162 (17.9%) patients with an MCD-like inflammatory state. Among MCD, there were 12 HHV8-MCD patients and 719 HHV-8-negative MCD patients, which included 139 asymptomatic MCD (aMCD) and 580 iMCD meeting clinical criteria. Of 580 iMCD patients, 41 (7.1%) met iMCD-TAFRO criteria, the others were iMCD-NOS. iMCD-NOS were further divided into iMCD-IPL (n = 97) and iMCD-NOS without IPL (n = 442). Among iMCD patients with first-line treatment data, a trend from pulse combination chemotherapy toward continuous treatment was observed. Survival analysis revealed significant differences between subtypes and severe iMCD (HR = 3.747; 95% CI: 2.112-6.649, p < 0.001) had worse outcome.
UNASSIGNED: This study depicts a broad picture of CD, treatment options and survival information in
China and validates the association between the CDCN\'s definition of severe iMCD and worse outcomes, requiring more intensive treatment.
UNASSIGNED: Beijing Municipal Commission of Science and Technology, CAMS Innovation Fund and National High Level Hospital Clinical Research Funding.