RT Book, Section A1 Dreyling, Martin A2 Kaushansky, Kenneth A2 Lichtman, Marshall A. A2 Prchal, Josef T. A2 Levi, Marcel M. A2 Press, Oliver W. A2 Burns, Linda J. A2 Caligiuri, Michael SR Print(0) ID 1121100532 T1 Mantle Cell Lymphoma T2 Williams Hematology, 9e YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 9780071833004 LK hemonc.mhmedical.com/content.aspx?aid=1121100532 RD 2024/03/29 AB SUMMARYMantle cell lymphoma is a distinct subtype of non-Hodgkin lymphoma with a pathognomonic chromosomal translocation t(11;14), leading to constitutive cyclin D1 overexpression. The clinical presentation usually is characterized by widespread disease, occurring more often in male patients older than age 60 years. Despite high initial response rates, early relapses occur frequently after conventional chemotherapy resulting in a median survival of only 3 to 5 years. However, 10 to 15 percent of patients present with a more indolent, chronic course. Dose-intensified treatment regimens containing cytarabine, rituximab, and autologous stem cell transplantation can achieve long-term remissions in patients fit enough to tolerate such aggressive therapy. For the majority of elderly patients, rituximab maintenance therapy can result in prolonged survival. Targeted approaches, including proteasome inhibitors, immunomodulatory drugs, and inhibitors of the B-cell receptor pathway, have proven highly efficacious in patients with relapsed disease and should be implemented in a multimodal treatment plan.