Efficacy of E-COD regimen in the treatment of HHV-8 negative multicentric Castleman disease

Castleman disease (CD) is a rare and heterogeneous disease whose treatment options and prognosis vary with clinical types. Multicentric Castleman disease (MCD) is characterized by poor prognosis, and effective treatment options are still being explored. This study aimed to determine whether the E-COD (E: etoposide 50 mg/m²/d, d1–3; C: phosphoramide 750 mg/m², d1, d3; O: vincristine 2 mg, d1; D: dexamethasone 10 mg/d, d1–5) regimen is effective in treating human herpesvirus-8 (HHV-8)-negative MCD. A group of patients diagnosed with MCD at Shengjing Hospital of China Medical University were treated with E-COD regimen. The effectiveness evaluation was conducted after four treatment cycles and follow-up for survival. A total of 101 patients were included in this study from January 2003 to December 2021, of whom 29 patients had HHV-8 negative MCD subtype. Seven HHV-8 negative MCD patients received four courses of E-COD chemotherapy. The complete response, partial response, and stable disease were 14.3%, 71.4%, and 14.3%, respectively. The follow-up ended on June 1, 2021. Two patients died, and five patients survived, with a survival period ranging from 2 to 11 years. This study suggests that the E-COD regimen is a safe, efficient, and affordable therapy option for HHV-8 negative MCD patients.