Haema 2013; 4(2):183-192
by Ioannis Apostolidis
Department of Hematology and Lymphomas and BMT Unit, “Evagnelismos” General Hospital, Athens, Greece
Follicular lymphoma (FL) is regarded as a prototypic model of low grade B cell lymphomas with strong dependence on the tumor microenvironment. At the genetic level the hallmark t(14;18) results in the constitutive overexpression of intact BCL2 protein that provides a survival advantage to the neoplastic cells, allowing them to revoke the default germinal center apoptotic program. The crosstalk between the FL B cells and the non-neoplastic microenvironment plays a fundamental role in maintaining tumor cell growth, promoting immune evasion, and supporting histologic evolution. Therapeutically, involved field radiation is the treatment of choice for localized stage I-II disease, while “watch and wait” remains the preferred strategy for patients with advanced stage asymptomatic disease. Patients with advanced stage symptomatic disease should be treated with chemotherapy + rituximab and the addition of maintenance rituximab is a reasonable option. Autologous and allogeneic stem cell transplantation are therapeutic options for patients with relapsed disease. Novel agents that target the microenvironment and the intracellular signalling pathways are expected to change the therapeutic landscape in the near future, and, currently, recruiting phase III clinical trials are questioning the role of chemotherapy as upfront therapy in patients with FL.