A prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone in relapsed/refractory iMCD. Issue 3 (23rd February 2022)

• Record Type: Journal Article
• Authors: Zhang, Lu; Zhang, Miao-yan; Cao, Xin-xin; Zhou, Dao-bin; Fajgenbaum, David C.; Dong, Yu-jun; Li, Jian
• Is Part Of: Leukemia & lymphoma. Volume 63:Issue 3(2022)
• Page Start: 618
• Page End: 626
• Publication Date: 2022-02-23
• Subjects: Idiopathic multicentric castleman disease -- bortezomib -- treatment; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2021.1999437
• Languages: English
• ISSNs: 1042-8194

Abstract

Abstract: Relapsed and refractory (R/R) idiopathic Multicentric Castleman disease (iMCD) is a clinical challenge with few treatment options. In this first multicenter, prospective trial which implemented the recently published CDCN response criteria, we evaluated the efficacy and safety profiles of bortezomib-cyclophosphamide-dexamethasone (BCD) regimen in 24 R/R iMCD patients. By 6 months, 15 patients (62.5%) achieved overall treatment responses; four patients (16.7%) had stable disease and five patients (20.8%) suffered from progression of disease. Even when considering all patients, there were significant ( p < .05) improvements in median symptom score, hemoglobin, platelet count, C-reactive protein (CRP) erythrocyte sedimentation rate (ESR), IL-6, albumin, and immunoglobin G (IgG) after treatment. The regimen was well tolerated without grade 3 or higher adverse events. Estimated 1-year progression-free survival (PFS) and overall survival (OS) were 79% and 92%, respectively. BCD regimen is an effective and safe treatment option for R/R iMCD patients. This trial was registered at www.chictr.org.cn as # ChiCTR1800019342.