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East Montgomery Times

Wednesday, April 23, 2025

New Castleman disease subtype discovered after 45 years, aiding treatment and diagnosis

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Kevin B. Mahoney CEO | Hospital Of The University Of Pennsylvania

Kevin B. Mahoney CEO | Hospital Of The University Of Pennsylvania

Philadelphia, April 22, 2025—Researchers from the Perelman School of Medicine at the University of Pennsylvania have discovered a new subtype of Castleman disease, named "Oligocentric Castleman Disease" (OligoCD), marking the first major advancement in the classification of this disease in 45 years. This breakthrough, published in Blood Advances, could significantly aid in the diagnosis and treatment of patients who have not fit existing disease classifications.

"This discovery is a game-changer for Castleman disease patients," said David Fajgenbaum, MD, associate professor of Translational Medicine and Human Genetics at Penn and co-founder of the Castleman Disease Collaborative Network (CDCN). Fajgenbaum emphasized the importance of this finding for patients who were previously misclassified and subjected to over-aggressive treatments such as chemotherapy.

Initially described in 1956 by Dr. Benjamin Castleman, Castleman Disease (CD) involves abnormal lymph node growth with varying symptoms. It was historically divided into unicentric Castleman disease (UCD) and idiopathic multicentric Castleman disease (iMCD). Some patients, however, did not fit these categories, complicating their care.

The identification of the OligoCD subtype was achieved using the ACCELERATE registry, which aggregates data from patients worldwide to aid research and treatment efforts. The study analyzed 179 patients, revealing that those with OligoCD experience fewer and less severe symptoms than those with iMCD. This suggests that surgical removal of affected lymph nodes may be more appropriate than treatments used for iMCD, which include IL-6 inhibitors, immunosuppressants, and chemotherapies. Further research is needed to refine treatment guidelines and understand OligoCD development.

"ACCELERATE has consistently proven to be an invaluable tool in unlocking the mysteries of Castleman disease," said Josh Brandstadter, MD, PhD, director of clinical research at Penn’s Center for Cytokine Storm Treatment & Laboratory.

The study engaged several Castleman Disease Collaborative Network patients, whose experiences helped classify OligoCD. One such patient, Penny Deremer of CDCN, expressed gratitude for finally having a name for her condition.

The ACCELERATE natural history registry has received past funding from Janssen Pharmaceuticals, EUSA Pharma, LLC, which merged with Recordati Rare Diseases Inc., and the U.S. Food & Drug Administration.

For further information, contact Matt Toal at 267-600-4871 or matthew.toal@pennmedicine.upenn.edu.

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