What is Mantle Cell Lymphoma?

Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma that makes up about 3 to 10% of all non-Hodgkin lymphoma diagnoses. Mantle cell lymphoma develops from B lymphocytes, which are cells in the lymphoid tissue that make antibodies and protect against germs and infection. Mantle cell lymphoma specifically affects B lymphocytes in the mantle zone, which is an area on the edges of lymph node follicles. Mantle cell lymphoma often presents as an aggressive type of cancer, although the presentation varies from very indolent/slow growing to more aggressive. Mantle cell lymphoma commonly involves organs outside of the lymph nodes when it is diagnosed. The disease may spread through the lymphatic system or blood to other lymph nodes, the spleen, bone marrow, or can spread to any other organ including the gastrointestinal tract, kidney, or lung, skin and rarely the central nervous system. Mantle cell lymphoma also has a high risk of coming back with time.

Most cases of mantle cell lymphoma are caused by a genetic mutation in which DNA is transferred between chromosomes 11 and 14. This chromosome "translocation" of one gene next to another causes the overproduction of a protein called cyclin D1, which stimulates uncontrolled cell growth and duplication of the cell. This protein can often be found on the surface of mantle cells, as well as within the cells themselves, and can be a way to diagnose the disease. The cause of this "translocation" is currently unknown, but doctors can use a specific test which is known as fluorescence in situ hybridization (FISH) to identify this abnormality or chromosomal translocation (t11;14) to be certain that a patient's biopsy shows mantle cell lymphoma.