From the Guidelines
The treatment of Multicentric Castleman Disease (MCD) should prioritize rituximab as first-line therapy, potentially combined with etoposide in severe cases to control cytokine storm consequences, as recommended by the most recent guidelines 1. When considering treatment options for MCD, it's crucial to weigh the benefits and risks, especially in HIV-positive patients, where antiretroviral therapy (ART) should be initiated or optimized 1.
- Key considerations include:
- Rituximab's role as a first-line treatment, despite not being EMA or FDA approved for MCD, due to its association with improved outcomes and reduced risk of lymphoma development 1.
- The potential addition of etoposide to rituximab in severe cases to mitigate the effects of a cytokine storm, although this combination is not universally approved for MCD treatment 1.
- The use of combination chemotherapy regimens, such as R-CHOP, in cases refractory to rituximab-based treatments, highlighting the need for personalized and adaptive treatment strategies 1. Given the complexity and variability of MCD presentations, a tailored approach considering the patient's HIV status, disease severity, and potential for cytokine storm is essential, with rituximab and etoposide forming the cornerstone of initial management in many cases, as per the latest clinical practice guidelines 1.
From the Research
Treatment Options for Castleman Disease
- The treatment of Castleman disease (CD) depends on the clinical classification, which can be either unicentric (UCD) or multicentric (MCD) 2.
- For UCD, surgical resection is the standard treatment and can confer a cure rate approaching 100% 3, 4.
- In cases where surgical resection is not possible, neoadjuvant rituximab has shown promise in reducing tumor size and enabling less morbid surgical resection 3.
- For MCD, the treatment is more complex and often insufficient, but biologic agents such as rituximab, siltuximab, and tocilizumab have improved survival rates significantly 5.
- Anti-interleukin-6-directed therapies are highly effective in many idiopathic MCD (iMCD) patients, but additional therapies are required for refractory cases 6.
Treatment of Unicentric Castleman Disease
- Surgical resection is the most effective treatment for UCD, with a high cure rate 4.
- Radiotherapy can also achieve clinical response and cure in selected patients, but the results are controversial 4.
- Neoadjuvant rituximab can be used to reduce tumor size and enable less morbid surgical resection 3.
Treatment of Multicentric Castleman Disease
- Biologic agents such as rituximab, siltuximab, and tocilizumab have improved survival rates significantly in MCD patients 5.
- Anti-interleukin-6-directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases 6.
- The treatment of MCD remains complex and often insufficient, and more clinical trials are required to assess the efficacy and safety of each agent 5.