Dissecting follicular lymphoma: high versus low risk

Abstract

Progress in the management of follicular lymphoma (FL) has translated to improved outcomes, with most patients surviving a decade or more from the time of diagnosis. However, the disease remains quite heterogeneous and a substantial number of patients have more aggressive disease with short responses to therapy and/or transformation to higher-grade lymphomas. Given the lack of a single standard approach, it is important to understand sources of heterogeneity among patients that influence initial management, surveillance strategies, and overall prognosis. Most of the validated tools, such as the Follicular Lymphoma International Prognostic Index (FLIPI) and FLIPI-2, apply to the frontline setting, and there is an unmet need for prognostic tools in relapsed and refractory disease states. In particular, the number of prior treatment regimens may be less important than the duration of response to the most recent regimen and the type of prior therapy received. Furthermore, despite awareness of progressive genetic and epigenetic derangements and a growing appreciation of the microenvironment’s role in FL outcomes, there is no validated means of incorporating biologic data into clinical prognostic indices. This review highlights the current state of knowledge regarding risk stratification in FL.