Follicular lymphomas

Follicular lymphomas constitute 30% of all non-Hodgkin lymphomas. These lymphomas are characterized by at least partially follicular growth pattern, but diffuse areas may be present. The proportions of follicular or diffuse areas vary also from case to case, which seems to be associated with prognosis. Follicular lymphomas should not be divided into distinct subtypes, but rather shows a continuous gradation in the number of large cells. On the bases of this grading, three groups have been defined: grades 1–3. There is a consensus that grade 3 follicular lymphomas, namely grade 3b, should be discriminated from lower-grade cases. The cells of follicular lymphomas express surface immunoglobulin, more frequently IgM +/− IgD > IgG > IgA, B-cell-associated antigens, CD10+/−; they are CD5−, CD23−/+, CD43−, and CD11c−. Follicular lymphomas express bcl-2 proteins, which is useful in distinguishing reactive from neoplastic follicles. t(14;18) is present in 70–95% of follicular lymphomas, involving rearrangement of bcl-2 gene. Clinical behavior of follicular lymphomas is heterogeneous and differs according to the histologic grade and extension of disease. Moreover, the evaluation of these malignancies is conditioned by therapeutic decision, which is also determined by main prognostic factors. The International Prognostic Index for aggressive lymphomas is not optimal for follicular lymphomas. Conversely, the Italian Lymphoma Intergroup Index and, more recently, the Follicular Lymphoma International Prognostic Index (FLIPI), designed in pre-rituximab era, seem to correlate well with outcome. Several active therapeutic approaches from the “wait and watch” strategy to the allogeneic transplantation are available for management of patients with follicular lymphoma. Therapeutic decision is mostly conditioned by patient's characteristics, stage, histologic grade, tumor burden, and risk-predicting factors.