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Published on: February 28, 2014
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.
Background:
CD (Castleman disease) is a rare and heterogeneous condition that poses a diagnostic and management challenge. Classification of CD has changed dramatically in recent years. This study aims to describe our experience with diagnosis and treatment of CD at a center in Canada.
Research Design And Methods:
We conducted a retrospective study of our cohort of CD patients evaluated and treated at the University of British Columbia between 2016-2025.
Results:
A total of 23 patients with biopsy-confirmed CD were included. 7/23 had unicentric CD (UCD), 1/23 had Oligocentric CD (OligoCD), and 15/23 had multicentric CD (MCD). All MCD cases were idiopathic (iMCD) and of these, 3/15 had iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction, and organomegaly), 4/15 had iMCD-idiopathic plasmacytic lymphadenopathy (iMCD-IPL), and 8/15 had iMCD-not otherwise specified (iMCD-NOS). Median time to diagnosis was shortest for iMCD-TAFRO (1 month) and longest in iMCD-IPL (82 months). 100% of iMCD-TAFRO, 100% of iMCD-IPL, and 86% of iMCD-NOS cases treated with Siltuximab or Tocilizumab had at least a partial response.
Conclusions:
This study demonstrates the heterogeneous presentation, natural history, and response to treatment of CD in a North American center, and highlights the importance of awareness of the different subtypes of CD. Key limitations are its retrospective design and small sample size.
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