Recent Progress in the Clinical Understanding, Diagnosis, and Treatment of Castleman Disease

Kefei Cao

doi:10.54254/2753-8818/2025.SH25835

Theoretical and Natural ScienceOpen access

Recent Progress in the Clinical Understanding, Diagnosis, and Treatment of Castleman Disease

Research Article

Open Access

Recent Progress in the Clinical Understanding, Diagnosis, and Treatment of Castleman Disease

Kefei Cao ^1*

¹ China-Japan Union Hospital of Jilin University

^*Corresponding author: ckf0310@163.com

Published on 6 August 2025

TNS Vol.131

ISSN (Print): 2753-8826

ISSN (Online): 2753-8818

ISBN (Print): 978-1-80590-291-1

ISBN (Online): 978-1-80590-292-8

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Abstract

Marked by clinical and pathological heterogeneity, Castleman disease (CD) is a lymphoproliferative disorder classified into unicentric (UCD) and multicentric (MCD) types. Distinct subtypes of Castleman disease differ in pathogenesis, pathology, and prognosis, yet diagnosis and treatment remain challenging, requiring standardized approaches. This paper explores the classification, pathological characteristics, diagnostic methods, and treatment strategies of CD, with a focus on the clinical and pathological features of different subtypes. Through analysis of recent literature on clinical presentations, imaging, histopathological diagnostic criteria, and therapeutic outcomes, this study provides evidence-based guidance for clinical practice. The results show that CD can be histologically categorized into hyaline vascular (HV), plasmacytic (PC), and mixed variants. Besides, MCD is further subdivided into HHV-8-associated, POEMS syndrome-associated, and idiopathic (iMCD) subtypes. The diagnostic approach combines clinical presentation, imaging results, and pathological evidence while excluding infectious, autoimmune, and malignant disorders. Treatment for UCD primarily involves surgical excision with favorable prognosis, whereas MCD requires tailored regimens including antiviral therapy, immunosuppression, targeted agents, and chemotherapy. Despite their efficacy in achieving quick symptom relief, corticosteroids are predominantly used as ancillary agents due to the constraints imposed by their long-term adverse effects.

Keywords:

Castleman disease, Lymphoproliferative disorder, Diagnosis, Treatment options

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References

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[5]. Lomas, O.C., et al. (2021). The management of Castleman disease. British Journal of Haematology, 195(3), 328-337.

[6]. Otani, K., Inoue, D., Fujikura, K., et al. (2018). Idiopathic multicentric Castleman's disease: A clinicopathologic study in comparison with IgG4-related disease. Oncotarget, 9(6), 6691-6706.

[7]. Wang, G., et al. (2025). (18)F-FDG PET/CT metabolic parameters are correlated with clinical features and valuable in clinical stratification management in patients of Castleman disease. Cancer Imaging, 25(1), 12.

[8]. Otsuka, M., et al. (2025). Exploring the clinical diversity of Castleman disease and TAFRO syndrome: A Japanese multicenter study on lymph node distribution patterns. American Journal of Hematology, 100(4), 592-605.

[9]. Abramson, J.S. (2019). Diagnosis and management of Castleman disease. Journal of the National Comprehensive Cancer Network, 17(11.5), 1417-1419.

[10]. Fajgenbaum, D.C., et al. (2020). Insufficient evidence exists to use histopathologic subtype to guide treatment of idiopathic multicentric Castleman disease. American Journal of Hematology, 95(12), 1553-1561.

[11]. Fang, F., et al. (2024). Efficacy of bortezomib, cyclophosphamide, and dexamethasone for newly diagnosed POEMS syndrome patients. Therapeutic Advances in Neurological Disorders, 17, 17562864231219151.

References

[1]. Pierson, S.K., et al. (2025). Characterizing the heterogeneity of Castleman disease and oligocentric subtype: Findings from the ACCELERATE registry. Blood Advances, 9(8), 1952-1965.

[5]. Lomas, O.C., et al. (2021). The management of Castleman disease. British Journal of Haematology, 195(3), 328-337.

[6]. Otani, K., Inoue, D., Fujikura, K., et al. (2018). Idiopathic multicentric Castleman's disease: A clinicopathologic study in comparison with IgG4-related disease. Oncotarget, 9(6), 6691-6706.

[9]. Abramson, J.S. (2019). Diagnosis and management of Castleman disease. Journal of the National Comprehensive Cancer Network, 17(11.5), 1417-1419.

Cite this article

Cao,K. (2025). Recent Progress in the Clinical Understanding, Diagnosis, and Treatment of Castleman Disease. Theoretical and Natural Science,131,14-20.

Data availability

The datasets used and/or analyzed during the current study will be available from the authors upon reasonable request.

About volume

Volume title: Proceedings of ICBioMed 2025 Symposium: Interdisciplinary Frontiers in Pharmaceutical Sciences

ISBN: 978-1-80590-291-1(Print) / 978-1-80590-292-8(Online)

Editor: Alan Wang, Xiangdong Xue

Conference website: https://www.icbiomed.org/shanghai.html

Conference date: 11 September 2025

Series: Theoretical and Natural Science

Volume number: Vol.131

ISSN: 2753-8818(Print) / 2753-8826(Online)

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