Department of Hematology, Seoul St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Department of Hematology, Yeouido St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Korean J Intern Med. 2021 Mar;36(2):424-432. doi: 10.3904/kjim.2019.330. Epub 2020 Feb 24.
BACKGROUND/AIMS: Idiopathic multicentric Castleman disease (iMCD) comprises approximately 30% of all cases of Castleman disease. It is characterized by constitutional symptoms, enlarged lymph nodes at multiple anatomical sites, and laboratory test abnormalities, which are primarily related to the overproduction of interleukin 6 (IL-6). Siltuximab is a human-mouse chimeric immunoglobulin G1κ monoclonal antibody against human IL-6. In view of the limited treatment options for iMCD, this study aimed to evaluate the efficacy and safety of siltuximab in the management of this condition.
In this real-world retrospective study, we administered siltuximab to 15 patients with iMCD who previously received conventional chemotherapy and/or steroid pulse therapy. The median time to a durable symptomatic response was 22 days (range, 17 to 56). The serum hemoglobin and albumin levels and erythrocyte sedimentation rates significantly normalized after the first 3 months of siltuximab treatment. Lymph node involution, assessed using imaging, was relatively gradual, demonstrating a complete or partial response at 6 months.
On an average, the improvements in clinical, laboratory, and radiologic parameters of iMCD in responders were observed after one, three, and eight cycles of siltuximab treatment, respectively. Siltuximab demonstrated a favorable safety profile, and prolonged treatment was well-tolerated.
Despite the small sample size of the present study, the results are encouraging and demonstrate the potential of siltuximab as the first-line treatment of iMCD. Further large multicenter studies are needed to evaluate the clinical outcomes and adverse events associated with siltuximab.
背景/目的:特发性多中心 Castleman 病(iMCD)约占 Castleman 病所有病例的 30%。其特征为全身症状、多个解剖部位的淋巴结肿大和实验室检查异常,主要与白细胞介素 6(IL-6)的过度产生有关。西妥昔单抗是一种针对人 IL-6 的人源化鼠嵌合 IgG1κ 单克隆抗体。鉴于 iMCD 的治疗选择有限,本研究旨在评估西妥昔单抗在该疾病治疗中的疗效和安全性。
在这项真实世界的回顾性研究中,我们给 15 例先前接受过常规化疗和/或类固醇冲击治疗的 iMCD 患者使用了西妥昔单抗。持久症状缓解的中位时间为 22 天(范围 17-56 天)。西妥昔单抗治疗的前 3 个月,血清血红蛋白和白蛋白水平以及红细胞沉降率显著正常化。使用影像学评估的淋巴结退缩相对缓慢,在 6 个月时达到完全或部分缓解。
在应答者中,iMCD 的临床、实验室和影像学参数的改善分别在西妥昔单抗治疗 1、3 和 8 个周期后平均观察到。西妥昔单抗具有良好的安全性,延长治疗也耐受良好。
尽管本研究的样本量较小,但结果令人鼓舞,表明西妥昔单抗有作为 iMCD 一线治疗的潜力。需要进一步的大型多中心研究来评估西妥昔单抗相关的临床结局和不良反应。