Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a rare form of B-cell non-Hodgkin lymphoma. B cells are part of the immune system and are made in the bone marrow. If these cells become cancerous, they can turn into a type of cancer called lymphoma. Like all lymphomas, MCL affects the lymphatic system, which includes the lymph nodes, spleen and bone marrow. However, MCL is different because it often also affects the gastrointestinal (GI) tract, including the stomach and colon.

MCL starts when cancerous B-lymphocytes (a type of white blood cell) grow in an area of a lymph node called the mantle zone — a ring of cells that surrounds certain structures inside lymph nodes. In about 10–20% of people with MCL, the cancer doesn’t show up in the lymph nodes at first. Instead, it’s found in the blood and bone marrow. This is called the leukemic phase of MCL.

When the cancerous B cells start to divide too often, they can build up and cause the lymph nodes to swell. For a small number of people, MCL grows slowly. But in most cases, it is aggressive and needs immediate medical attention. As the disease spreads to more lymph nodes and the bone marrow, it reaches more advanced stages.

Mantle cell lymphoma symptoms

MCL is more likely to affect men and people over age 65. Symptoms depend on the areas of the body where the lymphoma develops and usually aren’t detected until the cancer is advanced. Symptoms may include:

• Swollen lymph nodes, especially in the neck, armpits, or groin — this is often the most noticeable symptom.
• Abdominal swelling or discomfort, which may be caused by an enlarged spleen.
• Extreme tiredness, night sweats and unexplained weight loss that gradually gets worse.
• Frequent fevers without an obvious cause.
• High white blood cell count when MCL spreads to the bone marrow.
• Low red blood cells, platelets and neutrophils, which are important for fighting infection.
• Headaches, dizziness or confusion — these may happen if the cancer spreads to the brain or spinal cord.

What does mantle cell lymphoma feel like?

Mantle cell lymphoma usually starts with painless swelling in the neck, armpit or groin. People with MCL might also feel very tired, lose weight without trying, have fevers or sweat heavily at night. Because it can affect the stomach and intestines, it may cause belly pain, bloating or make you feel full quickly. If it spreads to the bone marrow, it can lead to problems like low energy, frequent infections or easy bruising. These symptoms can be hard to notice at first and may seem like other common illnesses, so it’s important to see a doctor if they don’t go away.

Mantle cell lymphoma diagnosis

Generally, MCL patients may note swollen lymph nodes, feel bloating in the abdomen or have a high white blood cell count found during routine blood tests. Sometimes, MCL is found during a routine colonoscopy when colon tissue shows signs of cancer. To confirm MCL, doctors will perform a biopsy of a lymph node, bone marrow or gastrointestinal tissue, followed by special lab tests. The tests look for changes in a gene called TP53 and check for abnormal chromosomes in your cells. If many abnormalities are found, it could mean the MCL is more aggressive or may not respond as well to certain treatments.

This process is called risk stratification. It helps your care team decide which treatments are most likely to work for you and allows them to plan more personalized care. The goal is to give you the best possible outcome based on your unique test results.

Mantle cell lymphoma treatment

MCL is not considered curable, but recent advances in treatment have helped many patients live longer and better lives. People with low- or intermediate-risk MCL often do well for five to eight years or more before the disease returns. In rare cases, especially when the disease is slow-growing (indolent), low-risk and not causing symptoms, patients may not need treatment right away and can be closely monitored instead.

Each person's treatment plan is personalized and based on several factors, including the stage of the lymphoma (early or advanced), symptoms, overall health and any other medical conditions the patient may have. Treatment options include the following:

Radiation therapy

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 limited or localized stage MCL, without high-risk features.

Learn more about radiation therapy.

Targeted therapy

Targeted therapies are designed to interfere with, or target, cancerous cells or the cancer-causing genes that create them.

They are often taken as oral pills and work by blocking a specific enzyme in mantel cell lymphoma cells. This helps stop them from growing and spreading. Sometimes, these oral medications are used along with monoclonal antibodies, a type of immunotherapy, which are given through an IV. Patients taking oral treatments usually don’t need a port (a small medical device placed under the skin, usually in the chest) for IV medicines.

Some common side effects from these drugs may include, fatigue, headache, rash, easy bruising, palpitations, low neutrophil count and infections.

Learn more about targeted therapy.

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. This procedure is intense and generally reserved for younger, physically fit patients.

Recent research shows that autologous stem cell transplants are used less often to treat mantle cell lymphoma when BTK inhibitors are given with chemotherapy at the beginning of treatment. These drugs are then continued as maintenance therapy along with a monoclonal antibody, which helps keep the cancer from coming back. At MD Anderson, we do not recommend stem cell transplants for most patients with MCL.

Learn more about stem cell transplants and their side effects.

Chemotherapy

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 combination of chemotherapy and monoclonal antibody therapy may be used to treat previously untreated MCL. However, because chemotherapy can have serious short- and long-term side effects, weaken the immune system, and newer targeted therapies are showing strong results, most MCL patients now prefer chemotherapy-free treatments.

Chemotherapy may still be used if the symptoms are severe and need to be treated quickly to shrink the tumor(s). In some cases, targeted therapies are also being combined with chemotherapy for older or frail MCL patients.

Learn more about chemotherapy and its side effects.

Monoclonal antibodies

Monoclonal antibodies are a type of immunotherapy. This treatment targets specific markers on the surface of MCL cells. These treatments are often used with targeted therapy and chemotherapy. They have shown significant benefits in helping MCL patients stay in long-term remission.

A combination of immunotherapy and chemotherapy, called chemoimmunotherapy, may also be used, followed by maintenance therapy. This approach has helped improve the overall survival of patients with MCL.

Learn more about immunotherapy and its side effects.

CAR T cell therapy

CAR T cell therapy is a type of immunotherapy. T cells are immune system cells that help the body fight disease by attacking harmful or infected cells. In Chimeric Antigen Receptor (CAR) T cell therapy, a patient’s T cells are collected and genetically modified in a lab to target a specific protein on cancer cells. These modified cells are returned to the patient’s body, where they seek out and attack the cancer cells.

CAR T cell therapy is typically used for people with relapsed or refractory mantle cell lymphoma (meaning their cancer has returned or hasn’t responded to other treatments). It’s not used as a first-line treatment, but rather after other therapies have not worked. However, CAR T cell therapy isn't suitable for every patient with relapsed or refractory MCL because of the possible side effects. Before choosing this treatment, care teams will look at factors like the patient's overall health, other medical conditions and organ function, especially if other treatments have not worked.

Learn more about CAR T cell therapy  and its side effects

Leukapheresis

This procedure is used to remove white blood cells (leukocytes) from the blood. It’s often performed on people with very high white blood cell counts, which can occur in certain types of leukemia. It is also used to collect specific white blood cells for treatments or research, such as CAR T cell therapy or stem cell transplants. During the procedure, an intravenous (IV) catheter is placed, and blood is drawn so the excess white cells can be separated and removed. A high white blood cell count can cause serious problems, including bleeding, headaches and tumor lysis syndrome. Tumor lysis can happen when cancer treatment causes cancer cells to break apart quickly, releasing chemicals and toxins into your bloodstream faster than your body can get rid of them.

Leukapheresis doesn’t cure mantle cell lymphoma because it only removes cancerous white blood cells from the bloodstream, not from where the cancer starts, like the bone marrow or lymph nodes. It provides temporary relief from high white cell counts but doesn’t stop the cancer from growing. Long-term treatment requires chemotherapy, targeted therapy, immunotherapy or other cancer-specific treatments.