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Castleman disease is an uncommon lymphoprolifera­tive disorder that continues to pose clinical challenges. Although surgical resection remains the standard ther­apy for unicentric disease, the landscape for the man­agement of multicentric disease continues to evolve.

Rituximab monotherapy is the current mainstay of therapy, and novel agents targeting interleukin 6 repre­sent exciting new additions to the treatment armamen­tarium. Single-agent and combination chemotherapies as well as antiviral therapy provide adjunctive support, particularly in the setting of relapsed or refractory dis­ease.

The ongoing exploration of antiviral and novel strategies, such as proteasome inhibition, is warranted. The management of Castleman disease also requires careful attention to potential concomitant infections, malignancies, and associated syndromes.

From the Center for Lymphoma at the Massachusetts General Hospital Cancer Center (JDS, JSA), Harvard Medical School (JDS, ARS, JSA), and the Department of Pathology (ARS) at the Massa­chusetts General Hospital, Boston, Massachusetts.

Submitted March 31, 2014; accepted June 18, 2014.

Address correspondence to Jeremy S. Abramson, MD, Center for Lymphoma, Massachusetts General Hospital Cancer Center, 55 Fruit Street, Yawkey 9A, Boston, MA 02114; E-mail: [email protected]

No significant relationships exist between the authors and the com­panies/organizations whose products or services may be referenced in this article.

The authors have disclosed that this article discusses unlabeled/ unapproved uses of the drug tocilizumab for the treatment of Castleman disease. 


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