View source for Mantle cell lymphoma overview

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{{ Mantle cell lymphoma }}

{{CMG}}; {{AE}} {{AS}}
==Overview==
Mantle  cell lymphoma is a subtype of B-cell lymphoma, due to [[CD5_%28protein%29|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|cyclin D1]] due to a'''t(11:14)'''<ref>http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html</ref> [[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 into typical and blastic variants, depending on the microscopic histopathology. 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. Male are more commonly affected with mantle cell lymphoma than female. 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]], painless swelling in the neck,under arm, groin, thorax and abdomen, and chest pain, abdominal pain, and bone pain. 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]]. [[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 (non-inherited) genetic mutations in [[somatic cells]]. Mantle cell lymphoma may be classified into typical and blastic variants, depending on the microscopic histopathology.
==Causes==
The cause of mantle cell lymphoma has not been identified.
==Differential Diagnosis==
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]].
==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. Male are more commonly affected with mantle cell lymphoma than female. 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===

The most common symptoms of mantle cell lymphoma include [[fever]], [[weight loss]], [[night sweats]], [[fatigue]], [[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 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===
There are no chest X ray findings associated with 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.

==References==
{{Reflist|2}}
{{Hematology}}

[[Category:Blood disorders]]
[[Category:Types of cancer]]
[[Category:Disease]]

[[Category :Hematology]]
 
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