From the Guidelines
Follicular lymphoma is a type of non-Hodgkin lymphoma that is considered incurable in the majority of patients with current standard therapeutic approaches, but it is very treatable and many people live with it for many years with a good quality of life. It is the second most common lymphoid malignancy, representing 20% to 25% of all cases of non-Hodgkin’s lymphoma (NHL), and the most common of the indolent NHLs 1. This type of cancer affects the lymphatic system, specifically involving white blood cells called B lymphocytes that grow abnormally in the lymph nodes, forming clusters called follicles.
Key Characteristics
- It is a slow-growing cancer, which means it develops gradually over months or years
- Many patients have no symptoms when first diagnosed, though some may notice painless, swollen lymph nodes in the neck, armpit, or groin
- Other possible symptoms include fatigue, night sweats, unexplained weight loss, or fever
- Follicular lymphoma is generally not curable, but treatment options depend on how advanced the disease is and may include:
- Watchful waiting (for slow-growing cases without symptoms)
- Chemotherapy
- Immunotherapy like rituximab
- Radiation therapy
- Stem cell transplantation
Prognosis and Treatment
The prognosis and treatment of follicular lymphoma have improved in recent decades, with a median overall survival that now exceeds 18 years 1. However, there are patients with high-risk disease features that have inferior outcomes to current therapies. The incorporation of rituximab to chemotherapy regimens has become a widely accepted standard of care for first-line therapy for patients with follicular lymphoma 1. Maintenance and consolidation therapy with rituximab and radioimmunotherapy have also been associated with improved progression-free survival in patients experiencing response to first-line therapy.
Monitoring and Follow-up
Regular follow-up appointments will be important to monitor the condition over time, as follicular lymphoma is generally considered a chronic disease characterized by multiple recurrences with current therapies 1. The newest risk prognostication developed in follicular lymphoma, the m7-FLIPI, has incorporated mutational analysis of seven genes along with the clinical risk factors of the FLIPI and Eastern Cooperative Oncology Group (ECOG) performance status to improve upon prognostication of follicular lymphoma patients receiving first-line chemoimmunotherapy 1.
From the FDA Drug Label
- 1 Non–Hodgkin's Lymphoma (NHL) RITUXAN is indicated for the treatment of adult patients with: Relapsed or refractory, low-grade or follicular, CD20-positive, B-cell NHL as a single agent Previously untreated follicular, CD20-positive, B-cell NHL in combination with first line chemotherapy and, in patients achieving a complete or partial response to a rituximab product in combination with chemotherapy, as single-agent maintenance therapy
Follicular lymphoma is a type of non-Hodgkin lymphoma (NHL), specifically a low-grade or B-cell NHL that is CD20-positive 2.
From the Research
Definition and Characteristics of Follicular Lymphoma
- Follicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells 3, 4, 5.
- It is characterized by diffuse lymphadenopathy, bone marrow involvement, and splenomegaly, with extranodal involvement being less common 3, 4, 5.
- Cytopenias are relatively common, but constitutional symptoms of fever, night sweats, and weight loss are uncommon in the absence of transformation to diffuse large B cell lymphoma 3, 4, 5.
Diagnosis and Prognosis
- The diagnosis is based on histology from a biopsy of a lymph node or other affected tissue, with incisional biopsy preferred over needle biopsies 3, 4, 5.
- Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl-2 protein 3, 4, 5.
- The Follicular Lymphoma International Prognostic Index (FLIPI) uses five independent predictors of inferior survival: age >60 years, hemoglobin <12 g/dL, serum LDH > normal, Ann Arbor stage III/IV, and number of involved nodal areas >4 3, 4, 5.
Treatment and Management
- Observation continues to be appropriate for asymptomatic patients with low bulk disease and no cytopenias 3, 4, 5.
- For patients needing therapy, most patients are treated with chemoimmunotherapy, which has improved overall response rates, duration of response, and overall survival 3, 4, 5.
- Maintenance rituximab has been shown to be effective in both frontline and relapsed/refractory settings 6.
- Other treatment options, such as lenalidomide, kinase inhibitors, stem cell transplantation, and chimeric antigen receptor T cells, are also considered for recurrent disease 3, 4, 5.