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The following is a summary of “Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience,” published in the October 2024 issue of Hematology by Jitaru et al. 

Castleman disease (CD) is a rare lymphoproliferative disorder, classified as unicentric or multicentric (MCD). MCD is linked to cytokine dysregulation, primarily IL-6, and siltuximab is recommended as a frontline treatment for idiopathic MCD (iMCD). 

Researchers conducted a retrospective study to analyze disease response and survival in patients with iMCD treated with siltuximab in Greece and Romania. 

This retrospective cohort study included adult patients with iMCD treated with siltuximab in Greece and Romania from January 2017 to December 2022. The primary endpoint was the overall response rate (ORR), with secondary endpoints including survival and safety. Response assessments followed the CD Collaborative Network guidelines. Patients were monitored until death, loss to follow-up, or study conclusion (October 2023). 

The results showed that 48 patients with iMCD were included, with a mean age of 65 years, differing by country (Greece: 74 years, Romania: 54 years). Most patients were male (68.8%) and had received 1 prior line of therapy (75%). They received a median of 9 cycles of siltuximab. Response data were available for 38 patients, showing an ORR of 71.1%, with 55.3% achieving a complete response (CR) and 15.8% a partial response (PR). The 3-year overall survival rate was 74%, with a median survival of 123 months. Common adverse events (AEs) (> 5%) included elevated liver enzymes, anxiety, allergic reactions, and nausea/diarrhea. Serious AEs occurred in 16.7% of patients. 

The study concluded that siltuximab-based therapy was effective in treating iMCD in real-world settings in Greece and Romania. It represented the largest real-world analysis of siltuximab in European patients with iMCD to date. 

Source: thejh.org/index.php/jh/article/view/1343