Initial treatment of limited stage (I/II) follicular lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jonathan W Friedberg, MD
Jonathan W Friedberg, MD
- Professor of Medicine
- James P Wilmot Cancer Center, University of Rochester
- Peter M Mauch, MD
Peter M Mauch, MD
- Professor of Radiation Oncology
- Harvard Medical School
Follicular lymphoma (FL, previously called follicle center lymphoma) is the second most common type of non-Hodgkin lymphoma (NHL). It is the most common of the indolent NHLs defined as those lymphomas in which survival of the untreated patient is measured in years. (See "Classification of the hematopoietic neoplasms".)
Treatment of FL depends upon the stage of disease at presentation (table 1). Patients with localized (stage I) disease are candidates for radiation therapy, which is curative in approximately half of patients. In contrast, the treatment of advanced (stage III/IV) disease is not curative and focuses largely on symptom control with chemoimmunotherapy with or without radiation therapy. The management of patients with stage II FL is more variable, with some clinicians offering treatment similar to that used for stage I disease, and others offering treatment similar to that used for advanced stage disease. There is some evidence that stage II follicular lymphoma limited to two contiguous regions has a similar prognosis to stage I. (See 'Stage II FL' below.)
The initial treatment of limited stage FL is discussed here. The initial treatment of advanced stage FL and the management of relapsed or refractory FL are presented separately, as are the epidemiology, clinical presentation, pathologic features, diagnosis, and pathobiology of FL. Of importance, the recommendations presented here pertain to patients with histologic grade 1, 2, or 3a FL; patients with grade 3b FL are treated as aggressive lymphomas (eg, diffuse large B cell lymphoma). (See "Initial treatment of advanced stage (III/IV) follicular lymphoma" and "Treatment of relapsed or refractory follicular lymphoma" and "Clinical manifestations, pathologic features, diagnosis, and prognosis of follicular lymphoma" and "Pathobiology of follicular lymphoma", section on 'Introduction' and "Pathobiology of follicular lymphoma".)
The initial evaluation of a patient with non-Hodgkin lymphoma (NHL) must establish the precise histologic subtype, the extent and sites of disease, and the performance status of the patient. These investigations are important for determining the treatment strategy and for predicting outcome with the Follicular Lymphoma International Prognostic Index (FLIPI) or one of its variants. General approaches to the diagnostic work-up and staging of NHL are presented separately (table 1). (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Evaluation, staging, and response assessment of non-Hodgkin lymphoma".)
Once a diagnosis and pathologic grade of FL is confirmed, the pre-treatment evaluation both determines the extent of the disease and provides information about the individual's comorbidities that are likely to have an impact on treatment options. Of particular importance, information gathered from this evaluation is used to determine the patient's Follicular Lymphoma International Prognostic Index (FLIPI) (table 2) score, which determines whether the patient is at low, intermediate, or high risk for progression. (See "Clinical manifestations, pathologic features, diagnosis, and prognosis of follicular lymphoma", section on 'Follicular lymphoma IPI (FLIPI)'.)
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- PRE-TREATMENT EVALUATION
- INITIAL MANAGEMENT
- Radiation therapy (RT)
- - Radiation field
- - Radiation dose
- Chemoimmunotherapy or immunotherapy alone
- SPECIAL SITUATIONS
- Grade 3 FL
- Stage II FL
- Intrafollicular neoplasia ("in-situ") FL
- Primary intestinal FL
- Primary cutaneous FL
- EVALUATION OF RESPONSE TO THERAPY
- SURVEILLANCE FOR RELAPSE
- CLINICAL TRIALS
- HISTOLOGIC TRANSFORMATION
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS