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).On microscopic histopathological analysis, centrocytes and centroblasts are characteristic findings of follicular lymphoma. Follicular lymphoma may be classified according to WHO criteria into 3 subtypes: low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Three variants of follicular lymphoma include pediatric follicular lymphoma, primary intestinal follicular lymphoma, and other extranodal 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 with follicular lymphoma than men. Prognosis is generally poor and the 5 year survival rate of patients with follicular lymphoma is approximately 72-77%. 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 for the diagnosis of follicular lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetics studies, flow cytometry, immunohistochemistry, FISH, genetic testing, and immunophenotyping. CT, MRI, and PET may be helpful in the diagnosis of follicular lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of follicular lymphoma. Other diagnostic studies for the diagnosis of follicular lymphoma include bone marrow aspiration and biopsy, laparoscopy, and laparotomy. 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 subtypes: low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Three variants of follicular lymphoma include pediatric follicular lymphoma, primary intestinal follicular lymphoma, and other extranodal follicular lymphoma.
Pathophysiology
Genes involved in the pathogenesis of follicular lymphoma include BCL-2, and BCL-6. The progression to follicular lymphoma involves the microRNAs (miRNAs). On microscopic histopathological analysis, centrocytes and centroblasts are characteristic findings of follicular lymphoma.
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 with follicular lymphoma than men.
Screening
Screening for follicular lymphoma is not recommended.
Prognosis
Prognosis is generally poor and the 5 year survival rate of patients with follicular lymphoma is approximately 72-77%.
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 for the diagnosis of follicular lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetics studies, flow cytometry, immunohistochemistry, 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.
Other Imaging Studies
PET scan may be helpful in the diagnosis of follicular lymphoma.
Other Diagnostic Findings
Other diagnostic studies for the diagnosis of follicular lymphoma include bone marrow aspiration and biopsy, laparoscopy, and laparotomy.
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.