Lymphoma, follicular

Lymphoma, Follicular Lymphoma, follicular is a type of non-Hodgkin lymphoma (NHL) that arises from B-lymphocytes, a type of white blood cell. It is characterized by a slow progression and is considered an indolent form of lymphoma. This article provides a comprehensive overview of follicular lymphoma, including its pathophysiology, diagnosis, treatment, and prognosis.

Pathophysiology

Follicular lymphoma originates from B-cells in the germinal centers of lymph nodes. It is associated with a chromosomal translocation, t(14;18)(q32;q21), which leads to the overexpression of the BCL2 gene. This genetic alteration prevents apoptosis, allowing the B-cells to survive longer than normal, leading to the accumulation of malignant cells.

Genetic Mutations

In addition to the BCL2 translocation, other genetic mutations are often present in follicular lymphoma. These include mutations in the CREBBP, EZH2, and MLL2 genes, which contribute to the pathogenesis of the disease.

Clinical Presentation

Patients with follicular lymphoma often present with painless lymphadenopathy, which is the swelling of lymph nodes. Other symptoms may include fatigue, night sweats, fever, and weight loss, collectively known as "B symptoms."

Diagnosis

The diagnosis of follicular lymphoma is typically made through a combination of:

• Histopathological examination: A biopsy of the affected lymph node is performed to examine the tissue under a microscope.
• Immunophenotyping: Flow cytometry is used to identify the specific markers on the surface of the B-cells, such as CD10, CD19, CD20, and CD22.
• Genetic testing: FISH (fluorescence in situ hybridization) or PCR (polymerase chain reaction) can be used to detect the BCL2 translocation.

Staging

Follicular lymphoma is staged using the Ann Arbor system, which considers the number and location of affected lymph nodes and the presence of systemic symptoms.

Treatment

The treatment approach for follicular lymphoma depends on the stage of the disease and the presence of symptoms. Options include:

• Watchful waiting: For asymptomatic patients with early-stage disease.
• Radiation therapy: For localized disease.
• Chemotherapy: Regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) are commonly used.
• Immunotherapy: Rituximab, a monoclonal antibody targeting CD20, is often used in combination with chemotherapy.
• Targeted therapy: Newer agents such as lenalidomide and idelalisib are being used in certain cases.

Prognosis

Follicular lymphoma is generally considered incurable, but it has a relatively good prognosis compared to other types of lymphoma. The median survival is over 10 years, and many patients experience long periods of remission.

Research and Future Directions

Ongoing research is focused on understanding the molecular mechanisms of follicular lymphoma and developing new targeted therapies. Clinical trials are exploring the use of CAR T-cell therapy and other novel agents.

See Also

• Non-Hodgkin Lymphoma
• B-cell Lymphoma
• Immunotherapy

External Links

• [Lymphoma Research Foundation](https://www.lymphoma.org)
• [American Cancer Society](https://www.cancer.org)

NIH genetic and rare disease info

• NIH info on Lymphoma, follicular

Lymphoma, follicular is a rare disease.