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Published on: October 14, 2021

The clinical spectrum of Castleman's disease

Angela Dispenzieri1, James O Armitage, Matt J Loe

  • 1Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu

American Journal of Hematology
|July 14, 2012
PubMed

Insights

Castleman's disease (CD) is a rare lymphoproliferative disorder. Prognostic factors, particularly POEMS syndrome, significantly impact survival, enabling better disease categorization and future research into this condition.

Area of Science:

  • Hematology
  • Oncology
  • Rare Diseases

Background:

  • Castleman's disease (CD) is a rare lymphoproliferative disorder with a poorly understood natural history and broad clinical spectrum.
  • Limited large-scale studies exist on the long-term course and prognostic indicators of Castleman's disease.

Purpose of the Study:

  • To describe the natural history of Castleman's disease.
  • To evaluate the impact of clinical and laboratory variables on overall survival (OS).
  • To investigate the relationship between CD subtypes and POEMS syndrome in predicting outcomes.

Main Methods:

  • Retrospective analysis of clinical and laboratory data from 113 patients with CD.
  • Evaluation of prognostic factors influencing overall survival (OS) from diagnosis.
  • Categorization of CD based on disease type (unicentric vs. multicentric) and association with POEMS syndrome.

Main Results:

  • Overall survival rates at 2, 5, and 10 years were 92%, 76%, and 59%, respectively.
  • Multicentric CD (MCD) patients showed varied outcomes, with 32% meeting POEMS syndrome criteria.
  • Four distinct CD categories were identified with significantly different 5-year OS: unicentric CD (91%), MCD with osteosclerotic POEMS (90%), MCD without POEMS (65%), and MCD with non-osteosclerotic POEMS (27%).

Conclusions:

  • Castleman's disease exhibits a spectrum of disease activity and outcomes.
  • POEMS syndrome is a critical determinant of survival in multicentric CD.
  • Prognostic stratification into four categories provides a framework for understanding CD and guiding further research.

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