New Paradigms in the Management of Castleman Disease

Vollständiger Abstract

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Castleman disease (CD) comprises a heterogeneous group of rare lymphoproliferative disorders unified by characteristic lymph node histopathology but with substantial clinical, biologic, and therapeutic diversity. Over the past decade, major advances in disease classification, pathophysiologic understanding, and targeted therapy have reshaped the management of CD, particularly idiopathic multicentric CD (iMCD). The introduction of IL-6–directed therapy has transformed the management of iMCD by shifting treatment from empirical immunosuppression or cytotoxic chemotherapy toward a biologic-based approach, achieving durable disease control in most patients. Progress has also been made over the past several years in the anatomic, etiologic, and clinical classification of CD, enabling more individualized, severity-adapted treatment approaches across the disease spectrum. Emerging paradigms in CD management include the recognition of oligocentric CD (OligoCD) as an intermediate anatomic entity with distinct therapeutic implications, as well as the validation of idiopathic plasmacytic lymphadenopathy (IPL) as a clinically distinct subtype of iMCD. These developments underscore the importance of integrating disease distribution, subtype, and severity into therapeutic decision-making. In addition, the recent paradigm of CD management emphasizes early identification of refractory disease and timely escalation beyond biologic therapy. This review provides an updated overview of CD management, drawing on recent advances to discuss practical considerations in treatment selection across disease subtypes, as well as ongoing challenges and future directions.