Diffuse large B-cell lymphoma.

摘要: Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults accounting for 31% of all NHL Western Countries. Following, morphological, biological and clinical studies have allowed subdivision DLBCLs into morphological variants, molecular immunophenotypic subgroups distinct disease entities. However, a number cases still remain biologically clinically heterogeneous, which there are no clear accepted criteria subclassification; these collectively termed DLBCL, not otherwise specified (NOS). DLBCL-NOS occurs adult patients, with median age seventh decade, but range broad, it may also occur children. Clinical presentation, behaviour prognosis variable, depending mainly extranodal site when they arise. These malignancies present localized manner approximately 20% patients. Disseminated less frequent, one third patients systemic symptoms. Overall, aggressive potentially curable malignancies. Cure rate particularly high limited 5-year PFS ranging from 80% to 85%; advanced ≈ 50%. The International Prognostic Index (IPI) adjusted IPI (aaIPI) benchmarks DLBCL prognosis. First-line treatment based on individual score age, three major should be considered: elderly (>60 years, aaIPI=0-3); young low risk (<60 aaIPI=0-1); aaIPI=2-3). combination anti-CD20 monoclonal antibody rituximab CHOP chemotherapy, every 14 or 21 days, standard Recent randomized trials suggest that high-dose chemotherapy supported by autologous stem cell transplant (HDC/ASCT) used as upfront high-risk outside prospective trials. HDC/ASCT actually recommended who did achieve CR after first-line chemotherapy. Consolidation radiotherapy reserved bulky immunochemotherapy. Patients score, indicates increased LDH serum level involvement more than site, certain sites (a.e., testes orbit) receive CNS prophylaxis part treatment. considered therapy chemotherapy-sensitive relapse. Overall results cannot managed due comorbidity disappointing. New effective toxic drugs agents worth considering this specific broad group Several novel undergoing evaluation DLBCL; among other, immunomodulating (lenalidomide), m-TOR inhibitors (temsirolimus everolimus), proteasome (bortezomib), histone deacetylase (vorinostat), anti-angiogenetic (bevacizumab) being investigated