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INTRODUCTION

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SUMMARY

Diffuse 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.

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Acronyms and Abbreviations

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; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CR, complete remission; CytaBOM, cytarabine, bleomycin, vincristine, methotrexate (with leucovorin rescue); DFS, disease-free survival; DLBCL, diffuse large B-cell lymphoma; EBV, Epstein-Barr virus; EFS, event-free survival; EPOCH, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin; ESHAP, etoposide, methylprednisolone, cytarabine, cisplatin; FDG, 18-fluorodeoxyglucose; GCB, germinal center B-cell–like; GELA, Group d’Etude des Lymphomes de l’Adulte; GVHD, graft-versus-host disease; ICE, ifosfamide, carboplatin, etoposide; IFRT, involved-field radiation therapy; Ig, immunoglobulin; 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.

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*This chapter contains elements from the chapter in the 8th edition of Williams Hematology written by Michael Boyiadzis and Kenneth A. Foon.

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DEFINITION AND HISTORY

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Diffuse large B-cell lymphomas (DLBCLs) comprise a heterogeneous group of aggressive malignancies of large, transformed B cells which cause diffuse effacement of the normal lymph node structure. DLBCL has masqueraded under a variety of colorful but misleading monikers in early lymphoma classification systems, including “reticulum cell sarcoma,” and “diffuse histiocytic lymphoma,” as described in an excellent recent review of the history of the lymphomas.1 Distinct disease entities are distinguished based on morphologic, biologic, and clinical features as established by an international panel of experts on behalf of ...

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