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Mantle cell lymphoma (MCL)

This page was reviewed under our medical and editorial policy by

Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was reviewed on May 31, 2022.

Mantle cell lymphoma (MCL) is a rare cancer that affects the lymphatic system, the network of lymph nodes and organs that extends throughout your body and carries a fluid filled with disease-fighting cells (lymph). MCL is a subtype of non-Hodgkin lymphoma (NHL), a larger category of cancer that includes more than 70 types of lymphoma.

MCL specifically starts in B-cells, which are white blood cells found in your lymphatic system. The role of these B-cells is to prevent infections by identifying harmful germs so that other immune system cells know to attack them.

MCL tends to be widespread by the time it’s diagnosed. This cancer is usually found in areas and organs such as:

  • Lymph nodes
  • Bone marrow
  • Spleen
  • Gastrointestinal system, including the esophagus, stomach and intestines

The speed at which MCL grows and spreads can vary. Treatment is influenced by whether the cancer seems to be slow-growing or fast-growing. When MCL is slow-growing, your care team may recommend delaying treatment until it’s clear the cancer is spreading and causing problems. However, fast-growing cases of MCL often require aggressive treatment.

About 81,560 cases of NHL were diagnosed in the United States in 2021, according to the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program. MCL affects about 5 percent to 7 percent of NHL patients.

Causes and risk factors of MCL

In most cases, lymphoma originates from acquired rather than inherited genetic changes, or mutations. When changes within DNA—your genetic material—are passed down from a parent to a child, they are called inherited mutations. But during a person’s lifetime, DNA can be damaged by numerous factors, which sometimes result in cancer-causing mutations. MCL, like most lymphomas, tends to arise from acquired mutations within specific cells in the body.

Researchers have identified a genetic change within the DNA of chromosomes 11 and 14 that is thought to be responsible for causing MCL. The mutation causes cells to produce excessive amounts of a protein that spurs rapid cell growth and results in cancer.

Risk factors for developing MCL aren’t well defined. However, MCL most often affects patients older than age 60, especially men. Other risk factors shared by all types of NHL include being white and having a weakened immune system due to genetic syndromes, certain immune-suppressing medications or conditions such as human immunodeficiency virus (HIV).

Symptoms of MCL

You may not notice any obvious symptoms with MCL, which is why it has often spread by the time of diagnosis. When symptoms are present, they may include:

  • Lymph node swelling (the most common symptom)
  • Fever
  • Night sweats
  • Weight loss
  • Fatigue
  • Stomach problems, such as nausea, vomiting, indigestion, pain and bloating

Swelling and discomfort may also occur in different areas, such as the throat, liver or spleen.

Diagnosing MCL

To make a diagnosis, your doctor will likely start with a physical examination to feel for enlarged lymph nodes and other signs of cancer and ask questions about your medical history and symptoms.

To treat lymphoma properly, your care team has to establish the exact subtype of the cancer. This requires collecting a sample of the potentially cancerous tissue, with a procedure called a biopsy, and sending it to a laboratory for testing and analysis.

A biopsy to test for MCL typically involves surgery to remove a suspicious lymph node or, in some cases, a portion of it. Lymph node biopsies can be more or less invasive depending on the location of the cancer. For example, lymph nodes near the surface of the skin are easier to remove than those deeper within the body.

At the lab, a pathologist, who is an expert in diagnosing diseases, examines the specimen under a microscope and performs various tests to check for cancer and, if necessary, identify the specific type. Testing methods include immunohistochemistry, which determines whether the cells contain specific MCL markers such as genetic changes in chromosomes 11 and 14.

While a biopsy is the key to diagnosing lymphoma and MCL, other tests may help determine the severity of the cancer and locate where it has spread. These tests may include:

Treating MCL

If the lymphoma is considered slow-growing, you may not need immediate treatment. Fast-growing lymphomas, however, typically require a faster response.

One of the most common treatment options for MCL is a combination of chemotherapy and a drug called rituximab. Rituximab was designed to target a specific protein found on the outside of the B-cells affected by MCL and other types of lymphoma. Chemotherapy regimens used alongside rituximab tend to be aggressive and involve several different drugs.

  • Chemotherapy plus rituximab tends to be given to patients who have widespread disease at the time of their diagnosis.
  • For patients with minimal spread, which is rare, radiation therapy may be an option.

Younger, otherwise healthy patients may be treated with an aggressive chemotherapy regimen along with a bone marrow transplant. In this procedure, cells are removed from either the patient’s or a donor’s bone marrow and stored while the patient receives a high-dose chemotherapy regimen. After the treatment, the cells are infused back into the blood. Taking rituximab for three years may be part of the approach to try to keep the cancer from returning. This tends to offer the highest chance of remission, or staying cancer-free.

Patients who are older or who have other medical conditions may be unable to withstand aggressive chemotherapy regimens or a bone marrow transplant. Instead, the care team may recommend chemotherapy regimens involving only one drug, such as bendamustine, along with rituximab.

If first-line treatments are unsuccessful or the cancer comes back after treatment, more rounds of chemotherapy may be used with different drugs. Certain targeted therapies, including Imbruvica® (ibrutinib), Calquence® (acalabrutinib) or Brukinsa® (zanubrutinib), may also be an option for recurrent or treatment-resistant MCL.

Clinical trials

MCL can be difficult to treat, and treatments are unlikely to fully remove the disease. However, options continue to improve as more research and clinical trials are conducted. MCL patients may want to consider enrolling in a clinical trial, particularly if first-line treatments were unsuccessful or the cancer recurs. Clinical trials provide access to experimental therapies that may prove to be more appropriate than current standard-of-care options.

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