First new subtype of Castleman disease discovered in 45 years
A new study co-authored by Penn Medicine’s David Fajgenbaum expands the spectrum of the rare disorder, which will help diagnose and treat patients caught between existing classification systems.
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A newly identified subtype of Castleman disease will help diagnose and properly treat thousands of patients who have been caught between existing classification systems, marking the first major discovery of its kind in 45 years. “Oligocentric Castleman Disease” (OligoCD) has been found to be a distinct clinical entity, different from the two previously identified classifications of Castleman disease. The findings, which redefine the understanding of this rare immune disorder that affects an estimated 4,300 to 5,200 Americans, are published in Blood Advances by researchers from the Perelman School of Medicine.
David Fajgenbaum is an assistant professor of medicine at the Perelman School of Medicine at the University of Pennsylvania and associate director of patient impact in the Penn Orphan Disease Center. He also leads the Castleman Disease Research Program.
(Image: Courtesy of Penn Medicine)
“This discovery is a game-changer for Castleman disease patients,” says David Fajgenbaum, an associate professor of translational medicine and human genetics and co-founder of the Castleman Disease Collaborative Network (CDCN). “For decades, patients with OligoCD have been falling through the cracks, classified as having a different type of Castleman disease and being subjected to potentially over-aggressive treatments such as chemotherapy that come with very uncomfortable side effects. Now we can match these patients—about 15% of all Castleman cases—with the right treatments for their specific condition.”
Using the ACCELERATE registry—which combines medical data from hundreds of patients with CD so that researchers and physicians can better understand and treat CD—the research team of clinicians and hematopathologists analyzed 179 patients.
The study finds that OligoCD patients exhibit fewer and less severe symptoms than those with iMCD, suggesting that surgical removal of affected lymph nodes—effective for the milder UCD—may be more appropriate than the intense treatments used for idiopathic multicentric Castleman disease (iMCD). Therapies for iMCD include IL-6 inhibitors used for serious rheumatoid arthritis, immunosuppressants used for autoimmune disease and transplants, and chemotherapies. However, the team emphasizes the need for further research to refine treatment guidelines and a further understanding of how OligoCD develops, with ACCELERATE poised to provide ongoing insights.
“ACCELERATE has consistently proven to be an invaluable tool in unlocking the mysteries of Castleman disease,” says Josh Brandstadter, director of clinical research at Penn’s Center for Cytokine Storm Treatment & Laboratory. “Without the robust data from patients around the world, we would not have been able to redefine the CD spectrum with such clarity.”
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