Updates in Low Grade Lymphoma

Disease overviewFollicular lymphoma is generally an indolent cell lymphoproliferative disorder of transformedfollicular center cells. Follicular lymphoma (FL) is characterized by diffuse lymphadenopathy,bone marrow involvement, splenomegaly and less commonly other extranodal sites of involvement.In general, cytopenias can occur but constitutional symptoms of fever, nightsweats, and weight lossare uncommon.DiagnosisDiagnosis is based on histology of preferably biopsy of lymph node. Immunohistochemicalstaining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonalimmunoglobulin, as well as cytoplasmic expression of bcl‐2 protein. The overwhelming majority ofcases have the characteristic t(14;18) translocation involving the IgH/bcl‐2 genes.Risk stratificationThe Follicular Lymphoma International Prognostic Index prognostic model for FL uses fiveindependent predictors of inferior survival: age 60 years, hemoglobin <12 g/dL, serumLDH normal, Ann Arbor stage III/IV, number of involved nodal areas 4. The presence of 0, 1, 2, and ≥ 3 adverse factors defines low, intermediate, and high‐risk disease. With the use of moremodern therapies, outcomes have improved.Risk‐adapted therapyObservation continues to be adequate for asymptomatic patients with low bulk disease and nocytopenias, with no survival advantage for early treatment with either chemotherapy or rituximabalone. For patients needing therapy, most patients are treated with chemotherapy plus rituximab,which has improved response rates, duration of response and overall survival. Randomized studieshave shown additional benefit for maintenance rituximab both following chemotherapy‐rituximaband single agent rituximab. Experimental therapies as well as stem cell transplantation (SCT) areconsidered for recurrent disease