Originally published as JCO Early Release 10.1200/JCO.2005.07.155 on February 22 2005

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Diffuse Large B-Cell Lymphoma: Clinical and Biological Characterization and Outcome According to the Nodal or Extranodal Primary Origin

From the Departments of Hematology and Pathology, Hospital Clínic, Institut de Recerca Biomèdica August Pi i Sunyer, Barcelona, Spain

Address reprint requests to A. López-Guillermo, MD; Department of Hematology, Hospital Clínic, Villarroel 170, 08036 Barcelona, Spain; e-mail: alopezg{at}clinic.ub.es

PURPOSE: To study the main clinicobiologic features, response, and outcome of patients with diffuse large B-cell lymphoma (DLBCL) according to the primary site, lymph node, or different extranodal organs of the disease.

PATIENTS AND METHODS: We included 382 patients consecutively diagnosed with DLBCL in a single institution during a 13-year period. Morphology, immunophenotyping, proliferation index, differentiation profile, bcl-2/JH rearrangement, and clinical characteristics were analyzed according to the primary site of the lymphoma.

RESULTS: Sites of the disease were: lymph node, 222 cases (58%); Waldeyer's ring (WR), 42 (11%); and extranodal sites, 118 (31%), including GI tract in 45 cases. Primary extranodal cases, particularly GI, showed a bcl-6 expression more frequently than nodal cases. Patients with primary WR or GI lymphomas presented with early-stage disease, no marrow infiltration, normal serum lactate dehydrogenase, and low- to low/intermediate-risk international prognostic index (IPI) more frequently than the remainder. Complete response (CR) rate was 63%, with WR and GI lymphomas having a higher CR rate (85% and 80%, respectively) than the other groups. In the whole series, 5-year overall survival (OS) was 52%. Patients with WR or GI lymphomas showed better OS (5-year OS: 77% and 68%, respectively) than patients with nodal or other extranodal sites. In the multivariate analysis, IPI, bulky disease, and ß2-microglobulin were the main variables to predict OS; no nodal or extranodal site maintained their prognostic value.

CONCLUSION: In the present series, the primary site of disease was associated with particular clinicopathologic features and outcome, though the latter largely depended on other factors.

Supported by grants FIS-IP03/0473, Spanish Ministry of Health; "Red de Centros de Investigación del Cáncer y de Grupos para el Estudio de Linfomas, Instituto Carlos III–FIS", and the José Carreras Foundation Against Leukemia (EM-P/04 and CR-P/04).

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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