New variant of Castleman disease uncovered after a 45-year gap
In a groundbreaking discovery, researchers from the Perelman School of Medicine have identified a new subtype of Castleman disease called Oligocentric Castleman Disease (OligoCD). This rare immune disorder, which affects an estimated 4,300 to 5,200 Americans, has been a mystery for decades.
The research, led by the Castleman Disease Collaborative Network, was published in Blood Advances and has significant implications for the diagnosis and treatment of this condition.
OligoCD accounts for about 15% of all Castleman disease cases. Unlike idiopathic multicentric Castleman disease (iMCD), OligoCD patients exhibit fewer and less severe symptoms. For years, patients with OligoCD have been classified as having a different type of Castleman disease, subjecting them to potentially over-aggressive treatments.
The discovery of OligoCD will help diagnose and properly treat thousands of patients who have been caught between existing classification systems. Surgical removal of affected lymph nodes may be more appropriate for OligoCD than the intense treatments used for iMCD.
Josh Brandstadter, director of clinical research at Penn's Center for Cytokine Storm Treatment & Laboratory, highlights the importance of the ACCELERATE registry's data for the redefinition of the Castleman disease spectrum. ACCELERATE, an ongoing registry, has been instrumental in unlocking the mysteries of Castleman disease and providing ongoing insights into the understanding of OligoCD and its development.
The team emphasises the need for further research to refine treatment guidelines for OligoCD. This discovery is a significant step forward in the understanding and management of this rare immune disorder.
For more information, read the full story by Matt Toal on Penn Medicine News.
