Update and new approaches in the treatment of Castleman disease

Abstract

First described 60 years ago, Castleman disease comprises a rare and heterogeneous cluster of disorders, characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical disturbances. Although unicentric Castleman disease is curable with complete surgical excision, its multicentric counterpart is a considerable therapeutic challenge. The recent development of biological agents, particularly monoclonal antibodies to interleukin-6 and its receptor, allow for more targeted disease-specific intervention that promises improved response rates and more durable disease control; however, further work is required to fill knowledge gaps in terms of underlying pathophysiology and to facilitate alternative treatment options for refractory cases.

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Keywords:

• Castleman disease
• angiofollicular lymph node hyperplasia
• biologics
• siltuximab
• tocilizumab
• rituximab

Disclosure

SJH has received funding from Janssen-Cilag for the conduct of clinical trials and participation in advisory boards in relation to Castleman disease. The authors report no other conflicts of interest in this work.