Mantle cell 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
Mantle cell lymphoma is a subtype of B-cell lymphoma, due to CD5 positive antigen-naive pregerminal center B-cell within the mantle zone that surrounds normal germinal center follicles. Mantle cell lymphoma cells generally over-express cyclin D1 due to at(11:14)[1] chromosomal translocation in the DNA. Development of mantle cell lymphoma is the result of (non-inherited) genetic mutations in somatic cells. Mantle cell lymphoma may be classified according to the microscopic histopathology into 2 variants: typical and blastic variants. Mantle cell lymphoma may be classified into 2 subtypes: low grade and high grade. The cause of mantle cell lymphoma has not been identified. Mantle cell 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 follicular lymphoma. In 2015, the incidence of mantle cell lymphoma was estimated to be 1.29 cases per 100,000 individuals in the United States. The prevalence of mantle cell lymphoma is estimated to be 5 cases per 100,000 individuals in the United States. Mantle cell lymphoma is a rare disease that tends to affect elderly patients > 60 years. Males are more commonly affected with mantle cell lymphoma than females. The male to female ratio is approximately 4 to 1. Prognosis is generally poor, and the 5-year survival rate of patients with mantle cell lymphoma is approximately 50-70%. Depending on the cell type of the tumor, the prognosis may vary. The most common symptoms of mantle cell lymphoma include fever, weight loss, night sweats, fatigue, skin rash, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of mantle cell lymphoma include fever, rash, splenomegaly, hepatomegaly, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for mantle cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, genetic testing, FISH, PCR, and immunophenotyping. Presence of nodules and pleural effusion on chest xray may be suggestive of mantle cell lymphoma. CT, MRI, and PET scan may be helpful in the diagnosis of mantle cell lymphoma. Other diagnostic studies for the diagnosis of mantle cell lymphoma include bone marrow aspiration, lumbar puncture, colonoscopy, upper endoscopy, laparoscopy, and laparotomy. The predominant therapy for mantle cell lymphoma is chemotherapy. Adjunctive immune based therapy, radioimmunotherapy, and new biologic agents may be required.
Classification
Mantle cell lymphoma may be classified into 2 subtypes: low grade and high grade.
Pathophysiology
Development of mantle cell lymphoma is the result of genetic mutations in somatic cells. Mantle cell lymphoma may be classified according to the microscopic histopathology into 2 variants: typical and blastic variants.
Causes
The cause of mantle cell lymphoma has not been identified.
Differential Diagnosis
Mantle cell lymphoma must be differentiated from diffuse large B cell lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma(MALT), small cell lymphocytic lymphoma, and follicular lymphoma.
Epidemiology and Demographics
In 2015, the incidence of mantle cell lymphoma was estimated to be 1.29 cases per 100,000 individuals in the United States. The prevalence of mantle cell lymphoma is estimated to be 5 cases per 100,000 individuals in the United States. Mantle cell lymphoma is a rare disease that tends to affect elderly patients > 60 years. Males are more commonly affected with mantle cell lymphoma than females. The male to female ratio is approximately 4 to 1.
Risk Factors
There are no established risk factors for mantle cell lymphoma.
Screening
Screening for mantle cell lymphoma is not recommended.
Prognosis
Prognosis is generally poor, and the 5-year survival rate of patients with mantle cell lymphoma is approximately 50-70%. Depending on the cell type of the tumor, the prognosis may vary.
Diagnosis
Staging
According to the Lugano classification, there are four stages of mantle cell lymphoma based on the number of nodes and extranodal involvement.
Symptoms
Symptoms of mantle cell lymphoma include fever, weight loss, night sweats, fatigue, skin rash, chest pain, abdominal pain, bone pain and painless swelling in the neck, under arm, groin, thorax and abdomen.
Physical Examination
Common physical examination findings of mantle cell lymphoma include fever, rash, splenomegaly, hepatomegaly, peripheral lymphadenopathy, and central lymphadenopathy.
Laboratory Tests
Laboratory tests for mantle cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, genetic testing, FISH, PCR, and immunophenotyping.
Chest X ray
Presence of nodules and pleural effusion on chest xray may be suggestive of mantle cell lymphoma.
CT Scan
CT scan may be helpful in the diagnosis of mantle cell lymphoma.
MRI
MRI may be helpful in the diagnosis of mantle cell lymphoma.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of mantle cell lymphoma.
Other Imaging Studies
PET scan (positron emission tomography) may be helpful in the diagnosis of mantle cell lymphoma.
Other Diagnostic Studies
Other diagnostic studies for the diagnosis of mantle cell lymphoma include bone marrow aspiration, lumbar puncture, colonoscopy, upper endoscopy, laparoscopy, and laparotomy.
Treatment
Medical Therapy
The predominant therapy for mantle cell lymphoma is chemotherapy. Adjunctive immune based therapy, radioimmunotherapy, and new biologic agents may be required.
Surgery
Surgical intervention is not recommended for the management of mantle cell lymphoma.