New variant of Castleman disease identified after a gap of 45 years

In a groundbreaking discovery, a team from the Perelman School of Medicine has identified a new subtype of Castleman disease, called Oligocentric Castleman Disease (OligoCD). This marks the first major discovery of its kind in 45 years.

The research, published in the prestigious journal Blood Advances, was conducted by the "CANDLE Study Group." The team analysed 179 patients with Castleman disease using the ACCELERATE registry, a resource that has been instrumental in the research of OligoCD and Castleman disease.

OligoCD is a distinct clinical entity, separate from the two previously identified classifications of Castleman disease. Unlike idiopathic multicentric Castleman disease (iMCD), which requires intense treatments such as chemotherapies, IL-6 inhibitors used for serious rheumatoid arthritis, and immunosuppressants used for autoimmune disease, surgical removal of affected lymph nodes may be more appropriate for OligoCD.

This new subtype affects an estimated 4,300 to 5,200 Americans, and OligoCD patients exhibit fewer and less severe symptoms compared to those with iMCD. David Fajgenbaum, an associate professor of translational medicine and human genetics and co-founder of the Castleman Disease Collaborative Network (CDCN), stated that this discovery is a game-changer for Castleman disease patients.

Josh Brandstadter, director of clinical research at Penn’s Center for Cytokine Storm Treatment & Laboratory, emphasizes the value of ACCELERATE in unlocking the mysteries of Castleman disease. He notes that without the robust data from patients around the world, the redefinition of the Castleman disease spectrum would not have been possible with such clarity.

The research team also seeks a further understanding of how OligoCD develops. They emphasize the need for further research to refine treatment guidelines for OligoCD. This story was written by Matt Toal and can be read more at Penn Medicine News.