Mantle Cell Lymphoma: Treatment Options

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. 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.

Active Surveillance

For the subset of patients who do not yet have symptoms and who have a relatively small amount of slow-growing disease, “active surveillance” (also known as “watch and wait” and “watchful waiting”) may be an acceptable option. With this strategy, patients’ overall health and disease are monitored through regular checkup visits and various evaluating procedures, such as laboratory and imaging tests. Active treatment is started if the patient 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. 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. Chemotherapeutic treatment approaches used to treat MCL are:

• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)
• Bendamustine (Treanda) in combination with rituximab
• Hyper-CVAD (cyclophosphamide, doxorubicin, vincristine, dexamethasone alternating with high-dose methotrexate and cytarabine) +rituximab

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

Allogeneic stem cell transplantation (in which patients receive stem cells from a related or unrelated donor), may increase response times for selected younger patients whose disease has relapsed (returned after treatment).

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.

Many new drugs used alone or in combination are being studied in clinical trials as initial induction therapy for MCL, including acalabrutinib (ACP-196), venetoclax (Venclexta), ofatumumab (Arzerra), and temsirolimus (Torisel). Please view the Mantle Cell Lymphoma: Relapsed/Refractory fact sheet for information about treatments being evaluated for relapsed/refractory (disease no longer responds to treatment) MCL.

To learn about treatments under investigation for MCL, download the Mantle Cell Lymphoma Fact Sheet.