Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience.

IF 1.3 Q4 HEMATOLOGY Journal of hematology Pub Date : 2024-10-01 Epub Date: 2024-10-21 DOI:10.14740/jh1343
Ciprian Jitaru, Argyris Symeonidis, Sorina Badelita, Eirini Katodritou, Andrei Colita, Anastasia Mpanti, Anamaria Bancos, Bogdan Tigu, Petra Rotariu, Laura Urian, Ioana Rus, Delia Dima, Anca Bojan, Marc Damian, Vasiliki Labropoulou, Mihai Stefan Muresan, Despina Fotiou, Bogdan Fetica, Bobe Petrushev, Angela Dascalescu, Dimitra Dalampira, Sanda Buruiana, Catalin Constantinescu, Mihnea Zdrenghea, Meletios A Dimopoulos, Ciprian Tomuleasa, Evangelos Terpos
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Abstract

Background: Castleman disease (CD) is a very rare, non-malignant lymphoproliferative disorder that can be classified as unicentric or multicentric (MCD). MCD is associated with systemic symptoms, including organ dysfunction due to cytokine dysregulation, primarily interleukin-6 (IL-6). The anti-IL-6 monoclonal antibody siltuximab is recommended as a frontline treatment for idiopathic MCD (iMCD), but real-world data on its use in routine clinical practice are limited. This study aimed to assess disease response and survival outcomes in patients with iMCD treated with siltuximab therapy in real-world settings in Greece and Romania.

Methods: This retrospective cohort study included adult patients with iMCD treated with siltuximab in clinical practice across Greece and Romania between January 2017 and December 2022. The primary endpoint was overall response rate and secondary endpoints included survival and safety outcomes. Response assessments were performed according to the Castleman Disease Collaborative Network guidelines. Patients were followed until death, loss to follow-up or study conclusion (October 2023).

Results: Forty-eight patients with iMCD were included in the study. Mean age at baseline was 65 years, with significant age differences between patients from Greece (74 years) and Romania (54 years). The majority of patients were male (68.8%) and received one prior line of therapy (75%). Patients included in the study received a median of nine cycles of siltuximab. Response data were available for 38 patients. The overall response to siltuximab was 71.1%, with 55.3% of patients achieving a complete response, and 15.8% a partial response. The estimated overall survival rate at 3 years was 74% and the median survival was 123 months. The most common adverse events (> 5%) included elevated liver enzymes, anxiety, allergic reactions and nausea/diarrhea. Serious adverse events were experienced by 16.7% of the patients.

Conclusions: Our results suggest that siltuximab-based therapy is effective in treating iMCD in real-world settings in Greece and Romania. To our knowledge, this study represents the largest real-world analysis of siltuximab in European patients with iMCD so far.

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西妥昔单抗治疗特发性多中心卡斯特曼病:真实世界的经验。
背景:卡斯特曼病(CD)是一种非常罕见的非恶性淋巴细胞增生性疾病,可分为单中心型和多中心型(MCD)。MCD伴有全身症状,包括细胞因子(主要是白细胞介素-6(IL-6))失调导致的器官功能障碍。抗IL-6单克隆抗体西妥昔单抗被推荐作为特发性MCD(iMCD)的一线治疗药物,但在常规临床实践中使用该药物的实际数据却很有限。本研究旨在评估在希腊和罗马尼亚真实世界环境中接受西妥昔单抗治疗的特发性MCD患者的疾病反应和生存结果:这项回顾性队列研究纳入了2017年1月至2022年12月期间在希腊和罗马尼亚临床实践中接受西妥昔单抗治疗的iMCD成年患者。主要终点为总体反应率,次要终点包括生存率和安全性结果。反应评估根据卡斯特曼病协作网指南进行。对患者进行随访,直至死亡、失去随访机会或研究结束(2023 年 10 月):48名iMCD患者参与了研究。基线平均年龄为 65 岁,希腊患者(74 岁)和罗马尼亚患者(54 岁)的年龄差异显著。大多数患者为男性(68.8%),之前接受过一种疗法(75%)。参与研究的患者接受了中位数为九个周期的西妥昔单抗治疗。38名患者获得了应答数据。硅妥昔单抗的总体应答率为71.1%,其中55.3%的患者获得完全应答,15.8%的患者获得部分应答。估计3年总生存率为74%,中位生存期为123个月。最常见的不良反应(> 5%)包括肝酶升高、焦虑、过敏反应和恶心/腹泻。16.7%的患者出现了严重不良反应:我们的研究结果表明,在希腊和罗马尼亚的实际环境中,基于西妥昔单抗的疗法能有效治疗 iMCD。据我们所知,这项研究是迄今为止对欧洲 iMCD 患者使用西妥昔单抗进行的最大规模的真实世界分析。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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Journal of hematology
Journal of hematology HEMATOLOGY-
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