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New Type of Castleman Disease Uncovered After 45 Years

Research led by David Fajgenbaum of Penn Medicine broadens the range of a seldom-encountered condition, streamlining diagnosis and treatment for patients who fall outside current classification boundaries.

Uncovered: A Novel Variant of Castleman Disease Emerges After Four and a Half Decades
Uncovered: A Novel Variant of Castleman Disease Emerges After Four and a Half Decades

New Type of Castleman Disease Uncovered After 45 Years

In a groundbreaking development, a team of researchers from the Perelman School of Medicine has discovered a new subtype of Castleman disease, known as Oligocentric Castleman Disease (OligoCD). This discovery, published in the journal Blood Advances, marks the first major discovery of its kind in 45 years.

OligoCD is estimated to affect an estimated 4,300 to 5,200 Americans, accounting for approximately 15% of all Castleman cases. This rare disease is distinct from the two previously identified classifications of Castleman disease.

Josh Brandstadter, the director of clinical research at Penn's Center for Cytokine Storm Treatment & Laboratory, emphasizes the importance of further research to refine treatment guidelines and gain a better understanding of how OligoCD develops.

The research was conducted using the ACCELERATE registry, which combines medical data from hundreds of patients with Castleman disease. ACCELERATE is poised to provide ongoing insights into the development and treatment of OligoCD. The study highlights ACCELERATE as an invaluable tool in unlocking the mysteries of Castleman disease.

OligoCD patients exhibit fewer and less severe symptoms than those with idiopathic multicentric Castleman disease (iMCD). The study suggests that surgical removal of affected lymph nodes may be more appropriate for OligoCD patients compared to the intense treatments used for iMCD, which include IL-6 inhibitors used for serious rheumatoid arthritis, immunosuppressants used for autoimmune disease, transplants, and chemotherapies.

The discovery of OligoCD may help match patients with the right treatments for their specific condition. OligoCD is considered a distinct clinical entity, and its identification marks a significant step forward in the understanding and management of Castleman disease.

The story about OligoCD is by Matt Toal. For more information, you can read the full article at Penn Medicine News. The study's primary authors are Dr. Frits van Rhee and colleagues from leading medical institutions.

Without the robust data from patients around the world, the redefinition of the Castleman disease spectrum would not have been possible with such clarity, according to Josh Brandstadter. The discovery of OligoCD underscores the importance of ongoing research and collaboration in the medical community.

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