Mantle cell lymphoma (MCL) is a B-cell type of lymphoma. It develops in the outer edge of a lymph node called the mantle zone. It makes up about 6% of all non-Hodgkin lymphomas. MCL occurs more often in men than in women, usually in adults over 50.
MCL is a unique type of lymphoma. MCL cells often look like a slow-growing (indolent) lymphoma under the microscope, but it behaves like an aggressive lymphoma and tends to grow quickly.
People with MCL usually have advanced stage disease (stage III or IV) when they are first diagnosed. The lymphoma has often spread to the lymph nodes, bone marrow, spleen and blood. MCL is sometimes found along the gastrointestinalgastrointestinalReferring to or having to do with the digestive organs, particularly the stomach, small intestine and large intestine. tract in the colon and stomach. When it is found here, it may be called lymphomatous polyposis.
In almost all cases of MCL, the lymphoma cells make large amounts of a protein called cyclin D1. This protein stimulates cell growth and allows the cancer cells to multiply and spread. The excess of cyclin D1 is usually caused by a translocation of chromosomes 11 and 14 [t (11;14)]. (A translocation occurs when segments of one chromosome break off and attach to a different chromosome.) Large amounts of cyclin D1 protein may play a part in the aggressive course of MCL.
MCL may appear a few different ways in a lymph node. The mantle zone type has a more indolent or less aggressive course than the blastblastAn immature bloodcell.-like type (blastoid variant), which has an aggressive course.
People with MCL often relapse after treatment. MCL has a poor long-term outlook and the median survivalmedian survivalThe period of time (usually months or years) after diagnosis or treatment at which half the people with a given disease will live longer and the other half will live less. for MCL is about 3–4 years.
Mantle cell lymphoma is usually treated like a diffuse large cell lymphoma. Although it can be a difficult lymphoma to treat, most people with MCL will get some type of treatment.
Chemotherapy is commonly used to treat MCL. Combinations of chemotherapy drugs are often used because MCL is usually advanced and there is widespread disease. Chemotherapy combinations that may be used include:
Bendamustine (Treanda) may also be used to treat people with MCL. It is given if the disease doesn’t respond to treatment, progresses during treatment or comes back after treatment (relapses).
Monoclonal antibodies are a type of biological therapy that is effective in treating certain types of NHL. Rituximab (Rituxan) is often used in combination with chemotherapy to treat MCL. The addition of rituximab to chemotherapy helps improve the response to chemotherapy treatment and the duration of remission.
Another biological therapy called bortezomib (Velcade) is a proteasome inhibitor. It may be given to people with mantle cell lymphoma who have relapsed or are no longer responding (refractory) to at least one therapy.
External beam radiation therapy is sometimes used for MCL. It may be given to the affected lymph node areas when there is early stage disease or as a palliative therapy to relieve symptoms.
A stem cell transplant (SCT) may be an option for some people who relapse after treatment or are no longer responding to treatment (refractory). There are some studies that suggest a SCT be used earlier in the course of MCL, so SCT may be offered to some younger and otherwise healthy adults with MCL when they are in a first remission. Whether this helps people with MCL live longer is still not known for sure.
People with MCL who relapse and are not suitable candidates for SCT, or those who relapse after a stem cell transplant, may be treated with rituximab or salvage chemotherapy regimens.
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