Rossini Bernardo, Cecchi Nicola, Clemente Felice, De Paolis Maria Rosaria, Hohaus Stefan, Innao Vanessa, Lucignano Mariano, Massaiu Roberto, Palumbo Giovanna, Rigolin Gian Matteo, Rossi Francesca Gaia, Verga Luisa, Guarini Attilio
Hematology and Cell Therapy Unit, IRCCS Istituto Tumori "Giovanni Paolo II" Bari, Italy.
Dipartimento di Oncologia ed Emato-Oncologia, Università degli studi di Milano, Milan, Italy.
Drugs Context. 2024 Mar 12;13. doi: 10.7573/dic.2023-9-4. eCollection 2024.
Castleman disease (CD) is a group of lymphoproliferative disorders that share common histopathological features yet have widely different aetiologies, clinical features and grades of severity as well as treatments and outcomes. Siltuximab is currently the only therapy approved by the FDA and EMA for idiopathic multicentric CD and is recommended as first-line therapy in treatment guidelines. Despite the extensive characterization of siltuximab treatment in clinical trials, available evidence from real-world practice is still scant. This collection of clinical experiences focuses on patients treated with siltuximab therapy, particularly regarding the idiopathic multicentric CD diagnostic work-up, and on treatment administration in patients with complex disease entering differential diagnosis with CD or concomitant diseases. Thus, these data help further characterize and improve the use of siltuximab in real practice in terms of effectiveness and safety of long-term administration as well as consequences of treatment interruption.
卡斯特曼病(CD)是一组淋巴增生性疾病,它们具有共同的组织病理学特征,但病因、临床特征、严重程度等级以及治疗方法和预后却大不相同。西妥昔单抗是目前美国食品药品监督管理局(FDA)和欧洲药品管理局(EMA)批准用于治疗特发性多中心型CD的唯一药物,并且在治疗指南中被推荐为一线治疗药物。尽管在临床试验中对西妥昔单抗治疗进行了广泛的特征描述,但来自实际临床实践的现有证据仍然很少。本临床经验集聚焦于接受西妥昔单抗治疗的患者,特别是关于特发性多中心型CD的诊断检查,以及患有复杂疾病且正在与CD或伴随疾病进行鉴别诊断的患者的治疗管理。因此,这些数据有助于进一步明确并改善西妥昔单抗在实际临床中的应用,包括长期给药的有效性和安全性以及治疗中断的后果。