REVIEW SERIES
Time for an individualized approach to first-line management of follicular lymphoma
Ferrata Storti Foundation
Guillaume Cartron1 and Judith Trotman2 Department of Hematology, University Hospital, Montpellier, France and 2Department of Hematology, Concord Hospital, Sydney, Australia
1
ABSTRACT
F
ollicular lymphoma is a heterogeneous B-cell lymphoma both in presentation and at progression. For most patients it is a chronic, relapsing indolent disease with overall survival expectations now potentially beyond 20 years. However, in a significant minority (~20%) who experience early progression or histological transformation after treatment, the disease no longer has an indolent behavior. This review looks at the development of prognostic indices, staging and therapies for follicular lymphoma, identifying where the data can, and cannot, guide the multidisciplinary team to determine an individualized approach to first-line therapy. A nuanced patient- and disease-specific approach is necessary to maximize disease response and survival while minimizing therapeutic toxicity.
Haematologica 2022 Volume 107(1):7-18
Introduction Follicular lymphoma (FL) is an indolent lymphoid neoplasm derived from germinal center B cells. Rapid therapeutic initiation is rarely required. With the oftenadvanced age of patients at diagnosis and the indolent nature of the disease, some patients will never need treatment. Almost half of patients with asymptomatic disease undergoing an accepted “watch and wait strategy” will not have commenced treatment 31 months after diagnosis.1 For symptomatic patients, the combination of a monoclonal antibody against CD20 (rituximab, obinutuzumab) with chemotherapy results in 80% survival at 10 years.2 Many of these patients will not experience disease relapse or die from their disease, even though FL remains the most common cause of death.3 The challenge for hematologists is to identify patients who need treatment and to define the most appropriate treatment for them considering their age, comorbidities and likelihood of subsequent relapse, to optimize both longevity and quality of life. While most patients are destined to have a prolonged survival, described as a “functional cure”, approximately 20% of patients will progress within the first 2 years of initiating treatment. These early failures, which frequently correspond with histological transformation into diffuse large B-cell lymphoma, constitute the true evolutionary turning point of this disease, accounting for more than 50% of patinets’ deaths in the first decade.3 Faced with difficulty in predicting an individual patient’s outcome before treatment initiation, it is important to implement a dynamic strategy that ensures the early identification of those patients who experience histological transformation in order to offer them innovative therapeutic strategies. The nuanced management of FL is now well charted. With our comprehensive knowledge of the disease, its evolution and patient-related factors, we are now able to discuss different therapeutic approaches with our patients. The recent arrival of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) challenges certain therapeutic choices. Here again, our evolving knowledge and a balanced and honest discussion with the patient should enable informed decision-making adapted to the patient’s life priorities.
Epidemiology and environmental factors
Correspondence: GUILLAUME CARTRON g-cartron@chu-montpellier.fr Received: June 23, 2021. Accepted: October 1, 2021. https://doi.org/10.3324/haematol.2021.278766
©2022 Ferrata Storti Foundation Material published in Haematologica is covered by copyright. All rights are reserved to the Ferrata Storti Foundation. Use of published material is allowed under the following terms and conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode. Copies of published material are allowed for personal or internal use. Sharing published material for non-commercial purposes is subject to the following conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode, sect. 3. Reproducing and sharing published material for commercial purposes is not allowed without permission in writing from the publisher.
FL is the most frequent indolent lymphoma, representing around 20% of all adult lymphomas in Europe, 30% in the USA and 10% in Asia and developing
haematologica | 2022; 107(1)
7
