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Diffuse large B-cell lymphomas (DLBCLs) are a heterogeneous group of tumors consisting of large, transformed B cells, accounting for approximately 25 to 30 percent of lymphoma cases. Incidence increases with age; the median age at presentation is in the seventh decade. The disease typically presents as a nodal or extranodal mass with rapid tumor growth associated with systemic symptoms. Approximately 50 to 60 percent of patients will present in an advanced stage. DLBCL is potentially curable with combination chemotherapy. For localized disease, six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) or three cycles of R-CHOP plus involved field radiation therapy is recommended, and for advanced DLBCL, six cycles of R-CHOP is appropriate. Whether dose adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) or dose-intensified R-CHOP is superior to standard R-CHOP is currently under investigation. High-dose chemotherapy with autologous stem cell transplantation is effective in relapsed or refractory DLBCL. Allogeneic stem cell transplantation should be considered, usually, as part of a clinical trial.

Acronyms and Abbreviations

Acronyms and abbreviations that appear in this chapter include: ABC, activated B-cell–like; ACVBP, doxorubicin (Adriamycin), cyclophosphamide, vindesine, bleomycin, prednisone; ALLO-HSCT, allogeneic hematopoietic stem cell transplantation; ASCT, autologous stem cell transplantation; BEAM, high-dose carmustine, etoposide, cytarabine, and melphalan; CDE, cyclophosphamide, doxorubicin, etoposide; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CHOPE or CHOEP, CHOP plus etoposide; CNOP, cyclophosphamide, mitoxantrone, vincristine, prednisone; CNS, central nervous system; CR, complete remission; CVAD, cyclophosphamide, doxorubicin, vincristine, dexamethasone; CytaBOM, cytarabine, bleomycin, vincristine, methotrexate (with leucovorin rescue); DFS, disease-free survival; DLBCL, diffuse large B-cell lymphoma; DSHNHL, Deutsche (German) High-Grade Non-Hodgkin’s Lymphoma Study Group; EBV, Epstein-Barr virus; EFS, event-free survival; EPOCH, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin; ESHAP, etoposide, methylprednisolone, cytarabine, cisplatin; FDG, fluoro-2-deoxyglucose; GCB, germinal center B-cell–like; GELA, Group d’Etade des Lymphomes de l’Adulte study; GVHD, graft-versus-host disease; ICE, ifosfamide, carboplatin, etoposide; I-CHOP, intensified CHOP; IFRT, involved-field radiation therapy; Ig, immunoglobulin; IL, interleukin; IVLBCL, intravascular large B-cell lymphoma; LDH, lactate dehydrogenase; MACOP-B, high-dose methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, bleomycin; m-BACOD, moderate-dose methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, dexamethasone; MOPP, mechlorethamine, vincristine, procarbazine, prednisone; OS, overall survival; PFS, progression-free survival; ProMACE, prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide; PTLD, posttransplantation lymphoproliferative disorder; R-CHOP, rituximab plus CHOP; R-EPOCH, rituximab plus EPOCH; R-ICE, rituximab plus ICE; VACOP-B, vincristine, doxorubicin, cyclophosphamide, etoposide, prednisone, and bleomycin; WHO, World Health Organization.

Definition and History

Diffuse large B-cell lymphomas (DLBCLs) are a heterogeneous group of aggressive lymphomas of large, transformed B cells. Consideration of morphology, biology, and clinical expression result in the subsets that are considered distinct disease entities as shown in Table 100–1 as proposed by the panel on classification of lymphoid tumors of the World Health Organization (WHO).1 The disease can arise de novo or may transform from a low-grade lymphoma, such as small lymphocytic lymphoma or follicular lymphoma. The history of the evolution of lymphoma diagnostic categories and their subtypes is discussed in Chaps. ...

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