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Br J Haematol. 2005 Apr;129(1):3-17.

The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care.

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  • 1Department of Medicine, Division of Infectious Disease, University of Washington School of Medicine, and The Program in Infectious Disease, Fred Hutchinson Cancer Research Center, Seattle, WA 98122, USA.


Fifty years ago, Dr Benjamin Castleman first described the unusual lymphoproliferative disorder that now bears his name. Over the subsequent decades, astute clinical and pathologic observations coupled with clever molecular biologic research have increased our understanding of the aetiology of Castleman disease (CD). This article proposes three broad CD variants based on both distinctive histopathology and clinical behaviour. The pivotal roles of infection with human herpesvirus 8 and interleukin-6 production in the development of CD are emphasized. Finally, the natural history of CD and the myriad of therapeutic options are reviewed in the context of a unified model of CD pathophysiology, and continued areas of uncertainty are discussed.

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