Mantle cell lymphoma is a rare form of NHL, constituting only about 6 percent of all NHL cases in the United States (approximately 3,000 cases per year). Although traditionally it has been considered to be an aggressive b-cell lymphoma, many physicians believe it is more appropriately classified as an indolent lymphoma. In any event, it most often affects men over the age of 60. Frequently, MCL is diagnosed at a later stage of disease and in most cases involves the gastrointestinal tract and bone marrow. The disease gets its name because mantle cell tumors are composed of cells that come from the “mantle zone” of the lymph node.
Treatments include watch and wait, chemotherapy regimens such as R-CHOP and the more HyperCVAD-MTX/AraC (hyper-fractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate and cytarabine) combined with rituximab (Rituxan). Stem cell transplantation has also shown promising results. Proteosome inhibitors, drugs that disrupt a molecular pathway that is critical for the elimination of proteins in both normal and cancer cells such as bortezomib (Velcade), may be used. Bendamustine (Treanda) in combination with other drugs such as rituximab (Rituxan) also may be used. Gemcitabine (Gemzar), a drug that interferes with cell growth, is being tested in MCL patients. It is also being studied in combination with bortezomib (Velcade). Other treatments under investigation include Bcl-2 directed therapies; rituximab (Rituxan); RIT; lenalidomide (Revlimid); thalidomide (Thalomid), and mTOR pathway inhibitors.
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