Commentary by UAMS’ van Rhee Featured in Blood Journal

By Linda Haymes

In the commentary, “Storming the Castle with TCP,” van Rhee and coauthor Katie Stone, then-director of the Immunotherapy Lab at the Myeloma Center, examined a report on the new treatment for newly diagnosed idiopathic multicentric Castleman disease (iMCD) patients.

Castleman  is a rare, incurable disease of lymph nodes that can cause fever, weight loss, fatigue and nausea.

The treatment used oral thalidomide, cyclophosphamide, and prednisone (TCP) to attack the disease through multiple mechanisms of action. It was reported by Lu Zhang and others in, “Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease.” Zhang wrote the TCP regimen showed promising results and safety and while more research is necessary, it is an important treatment option, particularly when siltuximab is unavailable.

“Significant progress has been made in recent years in the field of multicentric Castleman disease,” van Rhee wrote, noting several advancements. They include the establishment of the Castleman Disease Collaborative Network (CDCN), which classified MCD into distinct clinic-pathological entities followed by formal Castleman disease diagnostic and treatment guidelines, based on the severity of the disease, which spans a wide spectrum.

Treatment using siltuximab, while approved in both the United States and Europe, is not a cure, van Rhee wrote, and must be used lifelong because relapses have been reported when the therapy is stopped. It is also expensive and is not available in all parts of the world, he added.

Meanwhile, the new TCP regimen offers an all-oral and less cost-prohibitive treatment option in areas where access to siltumximab is limited.

“It is exciting to see a widely available, new treatment option for iMCD emerge; however many questions remain,” he wrote, adding that the progression of the rare, complex disease is still poorly understood.

“Ultimately, a thorough understanding of the disease is needed to develop more effective and better-targeted therapies for all iMCD patients,” van Rhee concluded.

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