Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 129, Issue 1, Pages 3-17Publisher
WILEY
DOI: 10.1111/j.1365-2141.2004.05311.x
Keywords
lymphoproliferative disease; Castleman disease; angiofollicular lymph node hyperplasia; human herpesvirus 8
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Funding
- NIAID NIH HHS [K23 1AI054162] Funding Source: Medline
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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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