Department of Hematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, People's Republic of China.
Zhejiang Provincial Clinical Research Center for Hematologic Diseases, Hangzhou, People's Republic of China.
Br J Haematol. 2023 Dec;203(5):803-806. doi: 10.1111/bjh.19000. Epub 2023 Jul 23.
Treatment options for idiopathic multicentric Castleman disease (iMCD) are currently limited, especially for patients who do not respond or are resistant to interleukin-6 inhibitors. For the first time, we innovatively designed a protocol using rituximab-bortezomib-dexamethasone (RVD) as first-line consolidation therapy in patients newly diagnosed with iMCD. Furthermore, we adopted a no-maintenance treatment strategy to simplify post-remission care. Five patients with iMCD were enrolled (including one with TAFRO syndrome) and underwent the RVD regimen, all of whom achieved partial response (PR) or better. After four cycles of RVD, three (60%) patients achieved PR, while one (20%) achieved a complete response. These five patients, who achieved PR or better, discontinued treatment but remained stable for a median follow-up of 11 months, with a duration of response of 7, 7, 10, 12 and 13 months, respectively. None of the patients experienced grade ≥3 adverse events during the observation period. Collectively, these findings demonstrated that the RVD regimen may be a promising treatment option for patients with iMCD. It was a safe and effective approach that resulted in lasting responses without the need for ongoing maintenance therapy.
特发性多中心 Castleman 病（iMCD）的治疗选择目前有限，特别是对于对白细胞介素-6 抑制剂无反应或耐药的患者。我们首次创新性地设计了一项方案，在新诊断为 iMCD 的患者中使用利妥昔单抗-硼替佐米-地塞米松（RVD）作为一线巩固治疗。此外，我们采用无维持治疗策略来简化缓解后的护理。共纳入 5 例 iMCD 患者（包括 1 例 TAFRO 综合征患者），接受 RVD 方案治疗，所有患者均获得部分缓解（PR）或更好的疗效。在接受 4 个周期的 RVD 治疗后，3 例（60%）患者达到 PR，1 例（20%）患者达到完全缓解。这 5 例达到 PR 或更好的患者停止治疗，但在中位随访 11 个月时仍保持稳定，缓解持续时间分别为 7、7、10、12 和 13 个月。在观察期间，没有患者发生≥3 级不良事件。综上所述，该研究结果表明，RVD 方案可能是 iMCD 患者的一种有前途的治疗选择。它是一种安全有效的方法，可产生持久的缓解，无需持续维持治疗。
