Diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults accounting for 31% of all NHL in Western Countries. Following, morphological, biological and clinical studies have allowed the subdivision of DLBCLs into morphological variants, molecular and immunophenotypic subgroups and distinct disease entities. However, a large number of cases still remain biologically and clinically heterogeneous, for which there are no clear and accepted criteria for subclassification; these are collectively termed DLBCL, not otherwise specified (NOS). DLBCL-NOS occurs in adult patients, with a median age in the seventh decade, but the age range is broad, and it may also occur in children. Clinical presentation, behaviour and prognosis are variable, depending mainly of the extranodal site when they arise. These malignancies present in localized manner in approximately 20% of patients. Disseminated extranodal disease is less frequent, and one third of patients have systemic symptoms. Overall, DLBCLs are aggressive but potentially curable malignancies. Cure rate is particularly high in patients with limited disease with a 5-year PFS ranging from 80% to 85%; patients with advanced disease have a 5-year PFS≈50%. The International Prognostic Index (IPI) and age adjusted IPI (aaIPI) are the benchmarks of DLBCL prognosis. First-line treatment for patients with DLBCL is based on the individual IPI score and age, and three major subgroups should be considered: elderly patients (>60years, aaIPI=0-3); young patients with low risk (<60years, aaIPI=0-1); young patients with high risk (