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SUMMARY

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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) are recommended, whereas for advanced-stage DLBCL, six cycles of R-CHOP are appropriate. 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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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 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 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 the World Health Organization (WHO; Table 9–1).2 The disease can arise de novo or may transform from an indolent lymphoma, such as a small lymphocytic lymphoma or follicular lymphoma.

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Table Graphic Jump Location
TABLE 9–1.*Diffuse Large B-Cell Lymphoma: Variants and Subtypes2
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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, carmustine (bischloroethylnitrosourea), etoposide, cytarabine (Ara C), and melphalan; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CR, complete remission; ...

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