Mantle Cell Lymphoma
Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells).
Mantle cell lymphoma (MCL) is a rare, B-cell NHL that most often affects men over the age of 60. The disease may be aggressive (fast growing) but it can also behave in a more indolent (slow growing) fashion in some patients. MCL comprises about five percent of all NHLs. The disease is called "mantle cell lymphoma" because the tumor cells originally come from the "mantle zone" of the lymph node. MCL is usually diagnosed as a late-stage disease that has typically spread to the gastrointestinal tract and bone marrow.
A diagnosis of MCL requires taking a small sample of tumor tissue, called a biopsy, and looking at the cells under a microscope. A blood test may also be necessary to measure the white blood cell count and certain proteins, which help to diagnose MCL. Other tests, such as a bone marrow biopsy and a computed axial tomography (CAT) scan may be used to confirm a diagnosis and to determine what areas of the body are involved by the cancer.
Overproduction of a protein called Cyclin D1 is found in more than 90 percent of patients with MCL. Identification of excess Cyclin D1 from a biopsy is considered a very sensitive tool for diagnosing MCL. One-quarter to one-half of patients with MCL also have higher than normal levels of certain proteins that circulate in the blood, such as lactate dehydrogenase (LDH) and beta-2 microglobulin. Measuring these and other proteins can help doctors determine how aggressive an individual patient's MCL is and may guide therapy decisions.
The type of treatment selected for a patient with MCL depends on multiple factors, including the stage of disease, the age of the patient, and the patient's overall health. For the subset of patients who do not yet have symptoms and who have a relatively small amount of slow growing disease, "watchful waiting" and monitoring the disease for progression may be an acceptable option. MCL is usually diagnosed once it has spread throughout the body, and the majority of these patients will require treatment. Initial treatment approaches for aggressive MCL in younger patients include combination chemotherapy, typically in combination with the monoclonal antibody rituximab (Rituxan), as first-line treatment, followed by autologous stem cell transplant (in which patients receive their own stem cells). HyperCVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone alternating with methotrexate and cytarabine) plus rituximab are recommended as aggressive induction therapy and are associated with durable remissions in newly diagnosed patients For older patients, chemotherapy followed by a prolonged course of rituximab alone, known as maintenance, is often recommended. A common chemotherapeutic treatment approach used to treat MCL is called R-CHOP, which combines rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. Bendamustine (Treanda) in combination with rituximab is another common first-line treatment option. Several additional intensified chemotherapy combinations are also used in combination with rituximab, particularly in younger patients.
Although high-dose chemotherapy followed by allogeneic (in which patients receive stem cells from a donor) stem cell transplantation is very intensive and causes various side effects, it may increase response times for selected younger patients.
Bortezomib (Velcade) is approved by the U.S. Food and Drug Administration for the treatment of MCL patients who have received at least one prior therapy. Recent studies with bortezomib show that the drug may be effectively combined with conventional chemotherapy.
On June 5, 2013, the U.S. Food and Drug Administration (FDA) approved lenalidomide (Revlimid) for the treatment of patients with mantle cell lymphoma (MCL) whose disease has relapsed or progressed after two prior therapies. Lenalidomide currently represents the first oral therapy for mantle cell lymphoma, which is a rare, B-cell non-Hodgkin lymphoma. Mantle cell lymphoma comprises about five percent of all non-Hodgkin lymphomas and is usually diagnosed as a late-stage disease that has typically spread to the gastrointestinal tract and bone marrow.
For complete details on the FDA approval, visit: http://www.fda.gov/Drugs/InformationOnDrugs/ApprovedDrugs/.
In November 2013, ibrutinib (Imbruvica) was approved to treat patients with Mantle Cell lymphoma who have received at least one prior therapy. For complete details about this approval, please visit: http://www.fda.gov/drugs/informationondrugs/approveddrugs/ucm374857.htm.
Many new drugs used alone or in combination are being studied in clinical trials for MCL, including idelalisib (GS-1101, formerly CAL-101), vorinostat (Zolinza), ofatumumab (Arzerra), everolimus (Afinitor), panobinostat, and temsirolimus (Torisel).
Clinical trials are crucial for identifying effective drugs and combinations for lymphoma patients. Because the optimal initial treatment of MCL is not clear and is such a rare disease, clinical trials are very important and will identify the best treatment options in this disease. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing email@example.com.
Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with the treatments they have received. Medical tests (such as blood tests and CAT scans) may be required at various times during remission to evaluate the need for additional treatment.
Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and the overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care.
Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.
A lymphoma diagnosis often triggers a range of feelings and concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma survivors (or caregivers) with volunteers who have gone through similar experiences.
LRF offers a wide range of resources that address treatment options, the latest research advances and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit the website at www.lymphoma.org or www.FocusOnMCL.org. You can also contact the Helpline at (800) 500-9976 or firstname.lastname@example.org.
View or order the following publications from our booklets/factsheets:
Professional staff members are available to answer your questions and provide individual support to you and your loved ones. Contact our Helpline, available Monday through Friday from 8:00am - 5:00pm Pacific Standard Time (PST). Call (800) 500-9976 or e-mail email@example.com.