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Time for an individualized approach to first-line management of follicular lymphoma
Guillaume Cartron1 and Judith Trotman2
1Department of Hematology, University Hospital, Montpellier, France and 2Department of Hematology, Concord Hospital, Sydney, Australia
ABSTRACT
Follicular 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 nec- essary to maximize disease response and survival while minimizing ther- apeutic toxicity.
Introduction
Follicular lymphoma (FL) is an indolent lymphoid neoplasm derived from germi- nal center B cells. Rapid therapeutic initiation is rarely required. With the often- advanced 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 com- menced treatment 31 months after diagnosis.1 For symptomatic patients, the com- bination 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 dis- ease, 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 ini- tiation, 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 thera- peutic 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
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
Ferrata Storti Foundation
Haematologica 2022 Volume 107(1):7-18
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
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