https://orcid.org/0000-0002-3853-5196
Key Points
Oligocentric Castleman disease validated as a distinct subtype with intermediate outcomes and management implications.
iMCD-IPL confirmed as a favorable Western subtype with unique responses, supporting subtype-specific treatment strategies.
Castleman disease (CD) is a heterogeneous group of lymphoproliferative disorders anatomically classified by distribution (unicentric [UCD], oligocentric [OligoCD], or multicentric [MCD]). HHV-8-negative MCD is called idiopathic MCD (iMCD) which includes clinical subtypes with varying phenotypes and responses: TAFRO (thrombocytopenia, anasarca, fever, renal dysfunction and/or reticulin fibrosis, and organomegaly), IPL (idiopathic plasmacytic lymphadenopathy), and NOS. OligoCD has recently emerged as an intermediate form between UCD and MCD with unclear clinical behavior, and IPL has not been validated in the Western cohort. We retrospectively analyzed 217 patients with CD evaluated at our institution between January 2004 and August 2024. Survival probabilities were compared using log-rank tests.57% had UCD, 20% had OligoCD, and 23% had iMCD. Patients with OligoCD and iMCD more frequently exhibited systemic symptoms than UCD. OligoCD and iMCD patients had significantly shorter EFS of 8.9 and 2.3 years than UCD (not reached; p < 0.001 and 0.02). Among iMCD subtypes, iMCD-IPL demonstrated a longer EFS compared to iMCD-TAFRO (p = 0.02) with no deaths during the follow-up periods.The results validate OligoCD and iMCD-IPL as new subtypes in this Western cohort. The survival outcome in OligoCD was intermediate between UCD and iMCD, with only a subset of OligoCD patients requiring systemic therapy. iMCD-IPL had a favorable survival outcome in this US cohort similar to what has been reported in non-Western countries. Tailoring treatment strategies to disease subtypes and vigilant monitoring of OligoCD for progression may improve survival outcomes in CD.
