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Oncologist. 2011;16(4):497-511. doi: 10.1634/theoncologist.2010-0212. Epub 2011 Mar 25.

Castleman's disease: from basic mechanisms to molecular therapeutics.

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  • 1Department of Investigational Cancer Therapeutics (Phase I Clinical Trials Program), MD Anderson Cancer Center, Unit 455, P.O. Box 301402, Houston, Texas 77030, USA.


Castleman's disease is a rare lymphoproliferative disorder in which there has been recent progress in elucidating underlying mechanisms with potential therapeutic implications. Unicentric Castleman's disease is an indolent condition that is often treated with local approaches. In contrast, patients with multicentric Castleman's disease (MCD) have a less favorable prognosis and require systemic treatment. Cytotoxic chemotherapy, with its attendant risk for toxicity, has been widely used to treat MCD, with variable efficacy. The discovery of putative etiologic factors and targets in MCD, particularly human herpes virus 8, CD20, and interleukin (IL)-6, has been translated into the use of rituximab and anti-IL-6-based therapy, as well as antiviral agents. In this article, we review the current state of the art of our understanding of Castleman's disease and its treatment and we provide insight into future treatment strategies based on disease biology.

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