[Low grade lymphoma: research progress and questions about treatment]

Treatment options for follicular lymphoma (FL) are considered when patients have limited stage, low tumor burden advanced stage, and high tumor burden advanced stage disease. Although patients with limited stage FL are managed with radiotherapy (24-36 Gy), watchful waiting (WW), rituximab monotherapy, and rituximab combined with chemotherapy need to be evaluated. In patients with low tumor burdens, WW is regarded as the standard management. The usefulness of rituximab monotherapy was also recently suggested. Rituximab combined with chemotherapy improved overall survival (OS) in patients with high tumor burdens and the optimal reference regimen might be R-CHOP or rituximab combined with bendamustine. As to efficacy, improved relapse-free survival, with rituximab maintenance, has been shown. In patients with primary macroglobulinemia, fludarabine improved both progression-free survival and OS as compared with chlorambucil. As to extra-nodal marginal-zone B cell lymphoma, the addition of rituximab to chlorambucil improves both the response rate and event-free survival. Large clinical trials of lenalidomide, idelalisib, and ibrutinib are now ongoing.