Castleman disease: application of modern classification in a Canadian center

Rebecca MacLeod1, Mark Trinder2, Liliana Wolak3

  • 1Department of Medicine, University of British Columbia, Vancouver, Canada.

Insights

Castleman disease (CD) presents heterogeneously, posing diagnostic challenges. This Canadian study found varied subtypes and treatment responses, emphasizing subtype awareness for better patient outcomes.

Area of Science:

  • Hematology
  • Rare Diseases
  • Oncology

Background:

  • Castleman disease (CD) is a rare, heterogeneous lymphoproliferative disorder.
  • Recent classification changes highlight diagnostic and management complexities.
  • Understanding CD subtypes is crucial for effective patient care.

Purpose of the Study:

  • To describe the diagnostic and treatment experiences with Castleman disease at a Canadian center.
  • To analyze the presentation, natural history, and treatment response across CD subtypes.

Main Methods:

  • Retrospective cohort study of 23 biopsy-confirmed Castleman disease patients.
  • Data collected from patients evaluated and treated at the University of British Columbia (2016-2025).

Main Results:

  • The cohort included unicentric CD (UCD), OligoCD, and multicentric CD (MCD) subtypes.
  • Idiopathic MCD (iMCD) subtypes included iMCD-TAFRO, iMCD-IPL, and iMCD-NOS.
  • Diagnosis times varied significantly by subtype, with iMCD-TAFRO being the fastest (1 month) and iMCD-IPL the slowest (82 months).
  • Targeted therapies (Siltuximab, Tocilizumab) showed high response rates (86-100%) across iMCD subtypes.

Conclusions:

  • Castleman disease exhibits diverse clinical presentations, natural history, and treatment responses.
  • Awareness of specific CD subtypes is essential for optimizing diagnosis and management.
  • Limitations include the retrospective design and small sample size, necessitating further research.
Abstract

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