Mantle Cell Lymphoma
Overview |
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- Mantle Cell Lymphoma is a histological subtype of Non-Hodgkin Lymphoma (NHL) that represents roughly 5% of NHLs. The disease typically affects the elderly.
Etiology |
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- The vast majority of mantle cell lymphomas harbor a translocation between Chromosome 11 and 14, t(11;14), which places the cell cycle regulator, Cyclin D1 under the promoter of the highly-expressed immunoglobulin heavy chain gene. This leads to continuous progression through successive cell cycles, contributing to neoplastic transformation.
Morphology and Immunophenotype |
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- The immunophenotype of neoplastic cells suggests an origin from B-cells in the mantle zone of secondary lymphoid follicles.
Clinical Consequences |
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- Patients typically present with painless lymphadenopathy at multiple sites and hepatosplenomegaly is also frequent. Mantle Cell Lymphomas are fairly aggressive and respond poorly to therapy, with patients displaying a median survival of roughly 6 years.