Mantle cell lymphoma (MCL) is a rare form of non-Hodgkin lymphoma. Like all lymphomas, MCL affects the lymphatic system, which includes the lymph nodes, spleen and bone marrow. MCL results from cancerous B-lymphocytes (a type of white blood cell) in the ‘mantle zone’ of lymph nodes, a ring of lymphocytes surrounding certain structures inside lymph nodes.
If these lymphocytes begin to divide too often, this can create an abnormal buildup of lymphoma cells that causes lymph nodes to become larger. While MCL grows slowly for a small number of people, it is generally aggressive and demands immediate attention. The stage of the disease increases as more lymph nodes and eventually the bone marrow becomes involved.
Mantle cell lymphoma symptoms
MCL is more likely to affect men and people over age 60. Symptoms depend on the areas of the body where the lymphoma develops and usually aren’t detected until the cancer is advanced. Symptoms include:
- Swelling in one or more lymph nodes, particularly in the neck, armpit or groin. This is the most obvious symptom of MCL.
- Frequent fevers
- Pressure or pain in the lower back
- Belly pain
- Night sweats
- Significant weight loss. MCL affecting bone marrow results in low red blood cell, platelet and neutrophil counts.
- Headaches, dizziness and confusion. These can occur if MCL spreads to the brain and spinal cord.
Many people don’t show visible symptoms in the disease’s early stages. MCL is often diagnosed after swollen lymph nodes lead to a blood test that shows a high number of white blood cells. A lymph node biopsy is then used to confirm if lymphoma cells are present.
Mantle cell lymphoma treatment
MCL cannot be completely cured in most people. However, prompt treatment can reduce the extent of the disease or even result in complete remission for years. A personalized treatment plan for MCL will consider various factors, including the lymphoma stage (early or advanced), symptoms and general health of the patient. Treatment options include the following:
Radiation therapy uses powerful, focused beams of energy to kill cancer cells. There are several different radiation therapy techniques. Doctors can use these to accurately target a tumor while minimizing damage to healthy tissue.
Radiation Therapy may be offered for early-stage MCL.
Targeted therapy drugs are designed to stop or slow the growth or spread of cancer. This happens on a cellular level. Cancer cells need specific molecules (often in the form of proteins) to survive, multiply and spread. These molecules are usually made by the genes that cause cancer, as well as the cells themselves. Targeted therapies are designed to interfere with, or target, these molecules or the cancer-causing genes that create them.
MCL targeted therapies make use of small molecule drugs.
Stem cell transplantation
A stem cell transplant (also known as a bone marrow transplant) is a procedure that replaces cancerous bone marrow with new, healthy bone marrow stem cells. Depending on the cancer, the healthy stem cells can come from a donor (an allogeneic transplant) or from the patient (autologous). Stem cell transplants are usually given after an intense round of chemotherapy that kills the patient’s existing bone marrow cells. Patients usually must stay in the hospital for three to four weeks after the transplant.
MCL patients can receive an autologous stem cell transplant. This can be a very effective but intense option, so it is typically offered only to younger, fit patients. It is usually followed by maintenance therapy that involves monoclonal antibody treatment for up to three years. Maintenance therapy is typically a long-term therapy designed to keep cancer in remission.
Chemotherapy drugs kill cancer cells, control their growth or relieve disease-related symptoms. Chemotherapy may involve a single drug or a combination of two or more drugs, depending on the type of cancer and how fast it is growing.
A chemotherapy regimen that combines chemotherapy and monoclonal antibody therapy may be used to treat advanced stage MCL. Chemotherapy alone used to be the main treatment option but due to the related side effects and relapse rate, other approaches are typically used.
The immune system finds and defends the body from infection and disease. Cancer is a complex disease that can evade and outsmart the immune system. Immunotherapy improves the immune system’s ability to eliminate cancer.
Immunotherapy has shown substantial potential for remission in MCL patients. A combination of immunotherapy and chemotherapy called chemoimmunotherapy may also be offered, followed by maintenance therapy.
CAR T cell therapy
Car T cell therapy is a type of immunotherapy. T cells are immune system cells that help the immune system respond to disease and directly kill diseased cells. In Chimeric Antigen Receptor (CAR) T-cell therapy, T cells are modified so they are able to recognize and attack cancer cells.
Treatments for mantle cell lymphoma are rapidly evolving and include chemotherapy-free treatment options and new forms of targeted therapy and immunotherapy, including new types of CAR T-cell therapy and bispecific antibodies.
Learn more: What to know about mantle cell lymphoma