TY - CHAP M1 - Book, Section TI - Mantle Cell Lymphoma A1 - Dreyling, Martin A2 - Press, Oliver W. A2 - Lichtman, Marshall A. A2 - Leonard, John P. PY - 2017 T2 - Williams Hematology Malignant Lymphoid Diseases 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - hemonc.mhmedical.com/content.aspx?aid=1148368750 ER -