Clinical Description
Lymphoma is a cancer of lymphocytes, which are white blood cells of the immune system that are located in lymph nodes throughout the body. Lymphoma is divided into two main subtypes: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Within the category of non-Hodgkin lymphoma, there are many different subtypes. Mantle cell lymphoma is a rare subtype that accounts for only 7% of all non-Hodgkin lymphomas. Mantle cell lymphoma is more common in people who are over the age of 60. Mantle cell lymphoma can cause enlarged lymph nodes throughout the body as well as enlarged liver and spleen. Some of the lymph nodes in the mouth, named Waldeyer ring, can also be involved. It can affect the bone marrow and the cells can also involve the circulating blood. It also commonly affects the gastrointestinal tract. Rarely, the skin and the brain may also be affected.
Symptoms of Mantle Cell Lymphoma
Symptoms for mantle cell lymphoma can be enlarged lymph nodes and/or the spleen. Mantle cell lymphoma can also cause fevers, chills, night sweats and/or unexplained weight loss. Gastrointestinal symptoms can also be common.
Risk Factors of Mantle Cell Lymphoma
There are no known risk factors for the development of mantle cell lymphoma.
How Mantle Cell Lymphoma is Diagnosed
Diagnosis is usually made with removing one the enlarged lymph nodes. Since they are many other lymphomas that can resemble this disease and the treatments are different, it is important to carefully review the lymph node under the microscope. Cyclin D1 is a protein which is over expressed in this type of lymphoma. FISH (fluorescence in-situ hybridization) is a test that can look at changes in the chromosomes of the cancer cells that are responsible for making this protein. Flow cytometry is sometimes to measure proteins present on the outside of the lymphoma cells.
Once the diagnosis is made under the microscope, CAT scans of the neck, chest, abdomen and pelvis are necessary to determine the degree of enlargement of the lymph nodes. Bone marrow biopsy and aspiration is usually performed to determine the degree of involvement of the bone marrow.
Evaluation of the gastrointestinal systems is sometimes necessary with possible colonoscopy/endoscopy, particularly if the patient is having any gastrointestinal symptoms. Evaluation of the function of the heart with an echocardiogram or MUGA scan can be necessary before any treatment since adriamycin, one of the chemotherapy drugs, can rarely affect heart function.
If there is concern for involvement of the brain, one treatment may become necessary is sampling of the fluid around the brain for evidence of lymphoma as well as administration of chemotherapy to the fluid around the brain. This fluid is accessed through a lumbar puncture.
Treatment Options for Mantle cell lymphoma at Tufts Medical Center
Mantle cell lymphoma is an aggressive lymphoma that usually requires intensive treatment. There are several different regiments for treatment of mantle cell lymphoma. One common regiment is HyperCVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternating with high-dose methotrexate and cytarabine, usually combined with rituximab). This regiment requires staying in the hospital during each cycle since the medications are given continuously. In older patients, R-CHOP (cyclophosphamide, vincristine, adriamycin, prednisone with rituximab) is frequently used, which can be given as an outpatient. Another treatment regimen of bendamustine with rituximab can also be effective. Autologous stem cell transplantation is usually recommended for eligible patients who achieve a remission on chemotherapy. If the disease returns despite chemotherapy, allogeneic stem cell transplantation may also be performed.
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