Mantle Cell Lymphoma: Treatment Options

There are a variety of factors a physician takes into consideration when choosing a treatment regimen, such as the stage of disease, the age of the patient and the patient’s overall health. MCL is usually diagnosed once it has spread throughout the body, and the majority of these patients will require treatment.  While mantle cell lymphoma is considered a difficult cancer to treat, tremendous progress has been made in the discovery of new treatments for the disease. Below are listed a summary of standard and approved therapies for mantle cell lymphoma.

Active Surveillance: Some patients present with asymptomatic, slow-growing disease. Such patients can be initially managed with "active surveillance."  With this strategy, patients' overall health and disease are monitored through regular checkup visits and various evaluating procedures, such a laboratory and imaging tests.  Active treatment is started if the patients begins to develop lymphoma-related symptoms or there are signs that the disease is progressing based on testing during follow-up visits.

Chemotherapy: 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 transplantation (in which patients receive their own stem cells), though rituximab is not specifically approved by the U.S. Food and Drug Administration (FDA) for MCL.  Combination chemotherapy containing cytarabine plus the immunotherapeutic monoclonal antibody rituximab are recommended as agressive induction therapy and are associated with durable remissions in newly diagnosed patients.  Consolidation high-dose chemotherapy followed by autologous stem cell is often utilized to prolong remission in younger, medically-fit patients.  For older or less fit patients, less intensive chemotherapy followed by a prolonged course of rituximab alone, known as maintenance therapy, is often recommended.  A common chemotherapeutic treatment approach used to treat MCL is called R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone).  Bendustamine (Treanda) in combination with rituximab is another common first-line treatment option.  Several additional intensive chemotherapy combinations are also used in combination with rituximab, particularly in younger patients.

Proteosome Inhibitors: These drugs disrupt a molecular pathway that is critical for the elimination of proteins in both normal and cancer cells. Bortezomib (Velcade) is a proteosome inhibitor that has been approved by the United States Food and Drug Administration (FDA) for the treatment of mantle cell lymphoma patients. Recent studies with bortezomib (Velcade) have demonstrated that the drug complements many conventional chemotherapy agents.

Transplantation:

A stem cell is an immature cell in the bone marrow that can develop into mature blood cells. These cells maintain a person’s blood cells, replacing older or damaged cells with newer ones. High-dose chemotherapy treatment can cause significant damage to stem cells in the bone marrow, compromising a person’s ability to renew their blood cells. The ability to transplant stem cells allows physicians to use higher doses of chemotherapy to treat the cancer than the body would normally tolerate, increasing the probability of killing cancer cells. If the chemotherapy is followed by an infusion of stem cells, these new stem cells can replace the cells in the bone marrow that were destroyed during the chemotherapy treatment.

There are two types of SCTs: allogeneic (patients receive stem cells from another person) and autologous (patients receive their own cells). In autologous stem cell transplantation, stem cells are donated by the patient and collected and frozen before the patient undergoes cancer treatment. After cancer treatment is given and the cancer cells are believed to be gone, the collected stem cells are reinfused back into the patient.

In allogeneic stem cell transplantation, the stem cell donor is not the patient, but is another person who is genetically similar—often a brother or sister. However, it is possible for the donor to be a person unrelated to the patient. After the patient has undergone chemotherapy and/or radiation therapy, the donor’s stem cells are infused into the patient. 

Reduced-intensity transplants (called non-myeloablative or mini-transplants) are procedures in which stem cells are received from an allogeneic donor, but the chemotherapy and/or radiation administered prior to the transplant is less intense (i.e., just enough to allow the body to accept the donor cells). The transplanted cells (the "graft") recognize the cancer as a foreign invader and activate immune cells to destroy it. Patients receiving reduced-intensity transplants may avoid some of the side effects seen with high-dose chemotherapy coupled with fully ablative allogeneic SCT.

Debate exists among researchers regarding which type of transplant (e.g., autologous versus allogeneic) is most efficacious and whether or when transplant should be used in the treatment of mantle cell lymphoma. High-dose chemotherapy coupled with SCT can be used to treat mantle cell lymphoma patients who have failed initial chemotherapy, but are responsive to a second chemotherapy regimen. Some researchers feel that allogeneic SCT is better for patients who have had a relapse and that autologous SCT should only be used to treat patients as part of initial therapy.

Treatments Under Investigation
Many new drugs used alone or in combination are being studied in clinical trials as initial induction therapy for MCL, including venetoclax (Venclexta), ofatumumab (Arzerra), and temsirolimus (Torisel).  

For additional information on these therapies, as well as treatments for mantle cell lymphoma that are currently under investigation, view or order your free copy of the Lymphoma Research Foundation's Mantle Cell Lymphoma Fact Sheet or Relapsed/ Refractory Mantle Cell Lymphoma Fact Sheet.

Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with their physician for any treatment updates that may have recently emerged.

Please note: It is critical to remember that today's scientific research is continuously evolving.