A prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone

in relapsed/refractory iMCD

1/31/2022 A study led by Dr. Zhang, a member of the CDCN SAB, recently studied a new treatment approach for iMCD patients not responding to previous options. They found a combination of bortezomib, cyclophosphamide, and dexamethasone (BCD) can be helpful in some patients and should be further evaluated in iMCD.


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 24R/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.

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