A newly recognised subtype of Castleman disease (CD), termed oligocentric CD (OligoCD), has been identified and characterised through the ACCELERATE natural history registry, offering clarity to a group of patients previously misclassified under existing categories.
Castleman disease, a rare group of lymphoproliferative disorders, has traditionally been divided into unicentric CD (UCD), involving a single lymph node region, and idiopathic multicentric CD (iMCD), which presents with widespread lymphadenopathy and systemic inflammation. However, a subset of cases has emerged that does not clearly fit either profile.
In a cohort study of 179 patients, an expert panel identified OligoCD as a distinct, intermediate clinical phenotype. These patients typically exhibit lymphadenopathy in a few localised regions, described as “oligocentric”, and display minimal inflammatory symptoms. On average, patients had involvement in three lymph node regions, fewer than in iMCD but more than the single site seen in UCD.
This discovery is significant as it helps resolve the diagnostic uncertainty faced by clinicians. Many OligoCD patients were previously misclassified as either UCD or iMCD, potentially affecting treatment approaches.
Researchers note that OligoCD more closely resembles UCD in terms of symptom severity but warrants its own clinical criteria for accurate diagnosis. Ongoing data collection through ACCELERATE aims to better understand the natural history and long-term risks associated with OligoCD.
The identification of OligoCD marks a vital step towards more precise diagnosis and tailored care for Castleman disease patients.
Reference
Pierson SK et al. Characterizing the heterogeneity of Castleman disease and oligocentric subtype: findings from the ACCELERATE registry. Blood Adv (2025) 9 (8): 1952–1965.
