Follicular lymphoma

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.

Follicular lymphoma is a form of non-Hodgkin lymphoma (see this term) characterized by a proliferation of B cells whose nodular structure of follicular architecture is preserved.

Prevalence of follicular lymphoma is estimated at about 1/3,000.

The median age at diagnosis is 60-65 years. The disease is extremely rare in children. Follicular lymphoma is located primarily in the lymph nodes, but can also involve the spleen, bone marrow, peripheral blood and Waldeyer's ring. The skin and central nervous system are affected in rare cases. Symptoms appear at an advanced stage of the disease and can include fever, night sweats and weight loss. At diagnosis, patients usually present with adenopathy and, in 50% of cases, splenomegaly.

In 85% of cases, follicular lymphomas are associated with a translocation t(14;18) (q32;q21), which activates the BCL2 gene encoding the BCL2 protein that is essential for some apoptosis processes.

Diagnosis is based on histological analysis of the adenopathy, a complete blood count, measurement of lactate dehydrogenase (LDH) and biopsy analysis of a lymph node. Examination using imagery (particularly a chest radiograph) is required. The observation of the translocation t(14;18) by polymerase chain reaction (PCR), in addition to suggested histological results, confirms the diagnosis.

Differential diagnoses include chronic lymphocytic B cell leukemia, diffuse large B cell lymphoma, mantle cell lymphoma and MALT lymphoma (see these terms).

If the lymphoma is localized it should be treated by radiotherapy. In the case of advanced stage lymphoma, chemotherapy with immuno-chemotherapy should be offered (rituximab combined with CHOP). In cases of chemosensitive relapse, treatment can be intensified.

The survival rate at five years is 80-90% and medial survival is approximately 10-12 years.

Visit the Orphanet disease page for more resources.

Last updated: 3/1/2010

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

• Copanlisib (Brand name: Aliqopa) - Manufactured by Bayer US LLC
  FDA-approved indication: For the treatment of adult patients with relapsed follicular lymphoma who have received at least two prior systemic therapies.
  National Library of Medicine Drug Information Portal
• PI3K-delta and PI3K-gamma inhibitor (Brand name: Duvelisib) - Manufactured by Verastem, Inc.
  FDA-approved indication: September 2018, PI3K-delta and PI3K-gamma inhibitor (Duvelisib) was approved for the treatment of adult patients with relapsed or refractory chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or follicular lymphoma after at least two prior therapies.
  National Library of Medicine Drug Information Portal
• Obinutuzumab (Brand name: Gazyva) - Manufactured by Genentech, Inc.
  FDA-approved indication: Indicated for the treatment of patients with follicular lymphoma (FL) who relapsed after, or are refractory to, a rituximab containing regimen. Also treatment of patients achieving at least a partial remission as well as the treatment of adult patients with previously untreated stage II bulky, II or IV follicular lymphoma.
  National Library of Medicine Drug Information Portal
  Medline Plus Health Information
• Rituximab and hyaluronidase human (Brand name: Rituxan Hycela) - Manufactured by Genentech, Inc.
  FDA-approved indication: Treatment of adult patients with relapsed or refractory, follicular lymphoma as a single agent, previously untreated follicular lymphoma in combination with first line chemotherapy and, in patients achieving a complete or partial response to rituximab in combination with chemotherapy, as single-agent maintenance therapy, non-progressing (including stable disease), follicular lymphoma as a single agent after first-line cyclophosphamide, vincristine, and prednisone (CVP) chemotherapy.
  National Library of Medicine Drug Information Portal

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