Castleman Disease: Insights from a Case Series and Literature Review

Abstract

Castleman disease (CD), a rare lymphoproliferative disorder with unicentric (UCD) and multicentric (MCD) subtypes, presents significant diagnostic and therapeutic challenges due to its clinical heterogeneity. This case series of four patients highlights rare and diverse clinical presentations of CD, including atypical anatomical sites and paraneoplastic pemphigus. Diagnostic challenges included differentiating CD from malignancies or autoimmune disorders, emphasizing the critical role of histopathology. UCD pathogenesis may involve follicular dendritic cell mutations, whereas MCD subtypes are driven by human herpesvirus (HHV)-8-associated viral interleukin-6 or idiopathic cytokine dysregulation. Treatment strategies varied, with surgery preferred for UCD and immunomodulators (siltuximab, rituximab) for MCD. Challenges such as limited standardized protocols in resource-constrained settings and biomarker variability underscore the need for individualized therapy. Emerging approaches, including cytokine-targeted therapies and bortezomib, show promise for refractory cases. This study reinforces the importance of multidisciplinary collaboration, early histopathological diagnosis, and long-term monitoring to optimize outcomes. By integrating clinical experiences with literature, it advocates for refined diagnostic criteria and context-specific therapeutic algorithms, urging further research to address gaps in managing this complex disease.

Keywords

case report - Castleman disease - unicentric CD - multicentric CD - human herpesvirus 8

Declaration of GenAI Use

Artificial intelligence was used exclusively to enhance language and readability; no data or content was generated solely by AI.

Patients' Consent

All figures in the article were obtained from the patient's medical record with proper consent. These are original images captured during the clinical evaluation.

Ethical Approval

This case report was granted institutional ethics committee clearance by the Institutional Ethics Committee, Government Medical College, Kozhikode, approval number GMCKKD/RP 2025/ IEC/26, written informed consent was obtained from the patient for publication.

Publikationsverlauf

Artikel online veröffentlicht:
18. Juli 2025

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