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A newly classified subtype of Castleman disease has emerged, promising to enhance the diagnosis and treatment options for thousands of patients historically misclassified within existing frameworks. This new condition, termed “Oligocentric Castleman Disease” (OligoCD), represents the first significant discovery in the field in 45 years. Researchers from the Perelman School of Medicine at the University of Pennsylvania have published their findings in Blood Advances, clarifying the nature of this rare immune disorder that affects an estimated 4,300 to 5,200 individuals in the United States.
“This finding marks a revolutionary shift for individuals living with Castleman disease,” said David Fajgenbaum, MD, an associate professor of Translational Medicine and Human Genetics at Penn and a co-founder of the Castleman Disease Collaborative Network (CDCN). “For years, patients diagnosed with OligoCD have received improper classifications, often leading to unnecessarily aggressive treatments, such as chemotherapy, that can cause significant side effects. Now, we can align these patients—who represent about 15 percent of all Castleman cases—with more suitable therapies tailored to their specific conditions.”
Castleman disease (CD), initially described in 1956 by Dr. Benjamin Castleman, includes a variety of conditions that lead to abnormal lymph node enlargement and a spectrum of symptoms ranging from mild to life-threatening. Traditionally, CD has been categorized into unicentric Castleman disease (UCD), which typically involves a single lymph node region with less severe symptoms, and idiopathic multicentric Castleman disease (iMCD), distinguished by widespread lymphadenopathy and severe inflammatory symptoms driven by cytokines. However, many patients’ symptoms do not fit neatly into these categories, complicating both diagnosis and treatment.
A New Subtype Identified
In their study, the research team utilized the ACCELERATE registry, which aggregates medical data from numerous CD patients, to gain a deeper understanding of the disease. They examined the cases of 179 patients, leading to the identification of OligoCD as a distinct subtype.
The results indicated that patients with OligoCD typically have fewer and less severe symptoms compared to those experiencing iMCD, suggesting that surgical intervention to remove affected lymph nodes—similar to the approach used for UCD—might be a more effective course of action than the more aggressive treatments reserved for iMCD. While therapies for iMCD currently include IL-6 inhibitors commonly used in treating severe rheumatoid arthritis, immunosuppressants for autoimmune disorders, and chemotherapy, the researchers stress the necessity for additional studies to establish precise treatment protocols and to further elucidate the mechanisms behind the development of OligoCD. The ACCELERATE registry is expected to play a crucial role in this ongoing research.
“The ACCELERATE registry has been instrumental in deciphering the complexities of Castleman disease,” noted Josh Brandstadter, MD, PhD, director of clinical research at Penn’s Center for Cytokine Storm Treatment & Laboratory. “The comprehensive data collected globally has allowed us to redefine the spectrum of Castleman disease with remarkable clarity.”
The collaborative effort also involved input from various patients within the CDCN, whose personal experiences shed light on existing classification shortcomings and motivated the initiative to formally categorize OligoCD.
Penny Deremer, a member of the CDCN and a patient with OligoCD at Penn Medicine, expressed her gratitude, stating, “I am immensely relieved to finally have a diagnosis that accurately reflects my experiences, as well as those of many other patients.”
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