Diagnosis and Management of Castleman Disease

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Jeremy S. Abramson
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Castleman disease is a heterogeneous nonmalignant lymphoproliferative disorder. Major distinctions include unicentric versus multicentric presentation; hyaline vascular, plasmacytic, or mixed pathology; and HHV8-associated (typically HIV-positive) versus idiopathic disease. At the NCCN 2019 Annual Congress: Hematologic Malignancies, Dr. Jeremy S. Abramson stated that rituximab is preferred as initial therapy for HHV8-positive disease, and chemotherapy can be added for patients with fulminant disease (antiretrovirals should always be used as well for those who are HIV-positive). Siltuximab is the preferred frontline therapy for idiopathic disease.

Disclosures: Dr. Abramson has disclosed that he receives consulting fees from AbbVie, Inc., Bayer HealthCare, Celgene Corporation, EMD Serono, Genentech, Inc., Janssen Pharmaceutica Products, LP, Karyopharm Therapeutics, Kite Pharma, and Roche Laboratories, Inc.

Correspondence: Jeremy S. Abramson, MD, MMSc, Massachusetts General Hospital Cancer Center, 55 Fruit Street, Boston, MA 02114. Email: jabramson@mgh.harvard.edu
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  • 1.

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  • 2.

    Gérard L, Michot JM, Burcheri S, et al. Rituximab decreases the risk of lymphoma in patients with HIV-associated multicentric Castleman disease. Blood 2012;119:22282233.

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    Zelenetz AD, Gordon LI, Abramson JS, et al. NCCN Clinical Practice Guidelines in Oncology: B-Cell Lymphomas. Version 5.2019. To view the most recent version, visit NCCN.org. Accessed October 15, 2019.

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  • 7.

    Zhang L, Zhao AL, Duan MH, et al. Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease. Blood 2019;133:17201728.

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