Newly diagnosed and relapsed follicular lymphoma: EHA Clinical Practice Guidelines for diagnosis, treatment, and follow-up

Abstract

Follicular lymphoma (FL) is the second most common lymphoma subtype in Europe, with a median age at diagnosis of 60–65 years. It is generally considered an indolent disease, and the median overall survival at diagnosis now likely exceeds 20 years. However, considerable heterogeneity exists. Diagnosis requires adequate biopsy material and a specialized hematopathologist. Staging and prognosis rely on positron emission tomography (PET)-computed tomography (CT). Prognostication at diagnosis is based on several validated clinical prognostic scores. Localized disease is usually treated with radiotherapy ± Rituximab. The treatment of disseminated disease includes active surveillance (or in selected cases Rituximab monotherapy) for low-tumor burden patients, and most often immunochemotherapy (or in selected cases chemo-free therapeutic options), ±antibody maintenance in high-tumor burden patients. For relapsed or refractory disease, treatment with Rituximab-Lenalidomide combination ± Tafasitamab or Epcoritamab from the first relapse and single-agent bispecific antibodies, chimeric antigen receptor T-cell therapy (CAR-T) cells, and Zanubrutinib-Obinutuzumab after second relapse should be prioritized over immunochemotherapy. Given the long natural history and risk of relapse, careful follow-up is an important part of patient management. Patients should be actively involved in decision-making. In this guideline, we present current evidence-based recommendations for diagnosis, staging, treatment, and follow-up.