RT Book, Section A1 Smith, Stephen D. A1 Press, Oliver W. 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 1121100395 T1 Diffuse Large B-Cell Lymphoma and Related Diseases T2 Williams Hematology, 9e YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 9780071833004 LK accessmedicine.mhmedical.com/content.aspx?aid=1121100395 RD 2024/04/24 AB SUMMARYDiffuse large B-cell lymphomas (DLBCLs) comprise a heterogeneous group of aggressive malignancies of large, transformed B lymphocytes.* DLBCL is the most common lymphoma in the world and accounts for approximately 25 to 30 percent of lymphoma cases in the United States. The incidence increases with age, with a median age at presentation in the sixth decade. The disease typically presents as a rapidly growing mass that may involve either lymph node or extranodal sites, and often is associated with systemic symptoms. Approximately 50 to 60 percent of patients will present with advanced stage, disseminated disease. DLBCL is curable with combination chemotherapy. For localized disease, either three cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) plus involved-field radiation therapy or six cycles of (R-CHOP) is recommended, whereas for advanced stage DLBCL, six cycles of R-CHOP is appropriate. A large phase III intergroup trial testing whether a novel infusional regimen consisting of dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH) is superior to standard R-CHOP has been completed, but the results not yet reported as of this writing. High-dose chemotherapy with autologous stem cell transplantation may be curative for patients with DLBCL that relapses after treatment with frontline chemotherapy.