Update: the investigation and management of follicular lymphoma

Abstract

Follicular lymphoma (FL) is a heterogeneous disease. For many it is experienced as a chronic, relapsing, indolent condition with long overall survival (OS). Most people affected have advanced disease at presentation; symptoms may include B symptoms (i.e. fever, night sweats and weight loss), fatigue and the local mass effect of lymph node enlargement. However, many people are asymptomatic at presentation. Some people are observed without treatment according to a 'watch and wait' policy (see section Management of patients with newly diagnosed FL). In contrast to this, over a period of many years, 20-30% of patients will die from refractory FL or following transformation of their disease to high-grade lymphoma. 1 Prognostic indices may help discriminate between risk groups (see section Prognostic factors in FL). Survival of people with FL has improved over the last 30 years. Single-institution series show up to 30% improvement in 5-year OS. 2,3 A USA population-based registry study of >14 000 patients between 1978 and 1999 showed an increase in median survival from 84 to 93 months. 4 Improvement in failure-free survival (FFS) was only seen following the introduction of anti-CD20 therapy given in combination with traditional chemotherapeutic approaches. Treatment plans for an individual person should be part of a long-term strategy and planned after multi-disciplinary team review with lymphoma specialist clinicians and nurses, specialist haematopathologists, radiologists and radiation oncologists. Numerous treatment modalities are available but some may compromise future choices. Risk of long-term complications, such as myelodysplastic syndromes, other secondary cancers, cardiac toxicity and effects on fertility must also be considered, given the extended and increasing survival of many people. Psychological support from clinical nurse specialists as well as other bodies, such as patient groups, is particularly important in the face of the chronic relapsing nature of this disease and the multiplicity of treatment choices available to the person affected and their physician. This document represents an update of the inaugural British Society of Haematology guideline, published in 2011, which now merits an update due to Significant developments in the understanding and therapy of the condition.