A national, multicenter, retrospective study of Castleman disease in China implementing CDCN criteria
Lu Zhang, Yu-jun Dong, Hongling Peng, Hao Li, Mingzhi Zhang, Huihan Wang, Qinhua Liu, Liping Su, Li-Ye Zhong, Wenjun Wu, Liang Huang, Xiaojing Yan, Lei Fan, Wenjiao Tang, Zhenling Li, Lin-tao Bi, Yan Li, Guangxun Gao, Li Gao, Ting-bo Liu, Yongqiang Wei, Yao Liu, Yu Li, Hui Zhou, Chunyan Sun, Wenbin Qian, Dehui Zou, Huilai Zhang, Kaiyang Ding, Xiaobo Wang, Ou Bai, Wenrong Huang, Bing Chen, Lin Yang, Jia Song, Dawen Gao, Tong Chen, Jun Luo, Shu-ye Wang, Liangming Ma, David C. Fajgenbaum, Li J
Abstract
Background 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. Methods 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. Findings 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. Interpretation 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. Fundings Beijing Municipal Commission of Science and Technology, CAMS Innovation Fund and National High Level Hospital Clinical Research Funding.