Follicular lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
Follicular lymphoma is defined as a lymphoma of follicle center B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern. Follicular lymphoma may be caused by translocation between chromosome 14 and 18 that results in the over expression of the ''BCL-2'' gene. The progression to follicular lymphoma involves the microRNAs (miRNAs). Follicular lymphoma may be classified according to WHO criteria into 3 groups: low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma. Follicular lymphoma must be differentiated from other diseases such as diffuse large B cell lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma (MALT), small cell lymphocytic lymphoma, and mantle cell lymphoma (MCL). The prevalence of follicular lymphoma increases with age. Women are more commonly affected. Prognosis of follicular lymphoma is generally poor. The median survival is around 10 years. According to the Lugano classification, there are four stages of follicular lymphoma based on the number of nodes, and extranodal involvement. The most common symptoms of follicular lymphoma include fever, weight loss, night sweats, skin rash, painless swelling in the neck, axilla, groin, thorax, and abdomen, and chest pain, abdominal pain, and bone pain. Common physical examination findings of follicular lymphoma include fever, rash, splenomegaly, peripheral lymphadenopathy, central lymphadenopathy, chest tenderness, abdominal tenderness, and bone tenderness. Laboratory tests of follicular lymphoma includes histologic confirmation, FISH, genetic testing, and immunophenotyping. CT and MRI may be helpful in the diagnosis of follicular lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of follicular lymphoma. The optimal therapy for follicular lymphoma depends on the stage at diagnosis, age, and prognostic scores. The predominant therapy for follicular lymphoma is chemotherapy. Adjunctive hematopoietic stem cell transplantation, and radioimmunotherapy may be required.
Classification
Follicular lymphoma may be classified according to WHO criteria into 3 groups: low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas, and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma.
Pathophysiology
Follicular lymphoma is defined as a lymphoma of follicle center B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern. Genes involved in the pathogenesis of follicular lymphoma include BCL-2, and BCL-6. The progression to follicular lymphoma involves the microRNAs (miRNAs).
Causes
Follicular lymphoma may be caused by translocation between chromosome 14 and 18 that results in the overexpression of the BCL-2 gene.
Differential Diagnosis
Follicular lymphoma must be differentiated from other diseases such as diffuse large B cell lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma (MALT), small cell lymphocytic lymphoma, and mantle cell lymphoma (MCL).
Epidemiology and Demographics
The prevalence of follicular lymphoma increases with age. Women are more commonly affected.
Prognosis
Prognosis of follicular lymphoma is generally poor. The median survival is around 10 years.
Diagnosis
Staging
According to the Lugano classification, there are four stages of follicular lymphoma based on the number of nodes, and extranodal involvement.
Symptoms
The most common symptoms of follicular lymphoma include fever, weight loss, night sweats, skin rash, painless swelling in the neck,under arm, groin, thorax and abdomen, and chest pain, abdominal pain and bone pain.
Physical Examination
Common physical examination findings of follicular lymphoma include fever, rash, spleenomegaly, peripheral lymphadenopathy, central lymphadenopathy, chest tenderness, abdominal tenderness, and bone tenderness.
Laboratory Findings
Laboratory tests of follicular lymphoma includes histologic confirmation, FISH, genetic testing, and immunophenotyping.
CT
CT scan may be helpful in the diagnosis of follicular lymphoma.
MRI
MRI may be helpful in the diagnosis of follicular lymphoma.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of follicular lymphoma.
Imaging Findings
There are no additional imaging findings for the diagnosis of follicular lymphoma.
Diagnostic Findings
There are no additional diagnostic findings for the diagnosis of follicular lymphoma.
Treatment
Medical Therapy
The optimal therapy for follicular lymphoma depends on the stage at diagnosis, age, and prognostic scores. The predominant therapy for follicular lymphoma is chemotherapy. Adjunctive hematopoietic stem cell transplantation, and radioimmunotherapy may be required.