IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE

Idiopathic multicentric Castleman disease (IMCD) is a rare and complex lymphoproliferative disorder characterized by excessive growth of polyclonal, reactive lymphoid cells in multiple lymph nodes, often associated with systemic symptoms such as fever, weight loss and fatigue.1 The incidence of this condition is extremely low (<5 cases per million people every year), but the disease can have a significant impact on patients’ quality of life. Diagnosis of IMCD can be challenging due to its similarity to other lymphoproliferative conditions and the need for a comprehensive workout and a multidisciplinary approach.2 The main therapeutic options currently available include the use of corticosteroids, chemotherapy, and targeted therapies, such as interleukin-6 inhibitors.3 Nonetheless, several patients require personalized and long-term treatment and research continues to seek new therapeutic strategies to improve outcomes. Prof. Arianna Di Napoli and Prof. Simone Ferrero will briefly discuss the fundamental aspects of diagnosis, clinical management and available therapeutic options for IMCD in 2025; then, they will also be available to answer participants’ questions, providing insights and clarifications on this pathology. The goal of this session is to provide a comprehensive and updated overview of the clinical management of IMCD, fostering a productive discussion between experts and participants to address the challenges still open in the care of this disease. Actually, despite advances in understanding the disease, many questions remain unanswered, making updates and discussion among experts crucial. References 1. Dispenzieri A, et al. Overview of Castleman disease. Blood. 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931. 2. Fajgenbaum, D. C. et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017 Mar 23;129(12):1646-1657. doi: 10.1182/blood-2016-10-746933. Epub 2017 Jan 13. 3. van Rhee, F. et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. doi: 10.1182/blood-2018-07-862334. Epub 2018 Sep 4.