Castleman disease: clinical features, pathology, and treatment outcomes in 12 cases - Report - MDSpire

Castleman disease: clinical features, pathology, and treatment outcomes in 12 cases

  • By

  • Yi Gong

  • Huiyang Liu

  • Yuanlong Li

  • Xin Wang

  • Jie Yuan

  • Jianming Wang

  • July 16, 2026

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Clinical Characteristics, Pathological Insights, and Treatment Results of Castleman Disease

Overview

This study presents a retrospective analysis of 12 patients with Castleman disease (CD), highlighting clinical manifestations, treatment outcomes, and the effectiveness of various therapeutic approaches.

Background

Castleman disease is a rare lymphoproliferative disorder with significant clinical heterogeneity and a low incidence rate. Accurate diagnosis and effective treatment are critical, especially given the challenges in distinguishing CD from other conditions in clinical practice. This study aims to provide real-world evidence from a Chinese cohort.

Data Highlights

CharacteristicDetails
Number of Patients12
Gender10 males, 2 females
Age Range20–67 years (median 45 years)
Diagnosis7 unicentric CD, 5 multicentric CD
Follow-up OutcomesAll patients alive, no recurrence in UCD cases

Key Findings

  • 7 patients diagnosed with unicentric CD (5 hyaline vascular type, 2 mixed type).
  • 5 patients diagnosed with multicentric CD (4 plasma cell type, 1 mixed type).
  • All patients tested negative for HHV-8 and HIV.
  • Complete surgical resection in UCD cases resulted in no recurrence.
  • MCD patients treated with R-CHOP achieved favorable outcomes with no recurrence during follow-up.
  • Oral TCD regimen was effective for a refractory MCD case.

Clinical Implications

Multicentric CD typically necessitates systemic therapy. In settings lacking access to anti-IL-6 therapies, R-CHOP may serve as a treatment option for idiopathic multicentric Castleman disease.

Conclusion

This analysis provides insights into the clinical management of Castleman disease.

Related Resources & Content

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  6. The ASCO Post — Using AI to Identify Treatment Options for Castleman Disease
  7. Frontiers in Medicine — Report of a Case of Renal Hilum Castleman Disease Complicated with Paget's Disease of the breast
  8. Using AI to Identify Treatment Options for Castleman Disease
  9. Case report: A rare case of retroperitoneal mixed type unicentric Castleman disease
  10. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms - PMC
  11. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease | Blood | American Society of Hematology
  12. Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease - Talaulikar - Internal Medicine Journal - Wiley Online Library
  13. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease - PMC
  14. Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial - PubMed
  15. Human Herpesvirus-8 Disease: Adult and Adolescent OIs | NIH
  16. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease - PMC
  17. Unicentric Castleman Disease of the Mesentery: A Systematic Review - PMC

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