Characteristics
Mantle cell lymphoma (MCL) is a rare type of non-Hodgkin lymphoma (NHL) that develops from B-lymphocytes (a type of white blood cells that produce antibodies) in the mantle zone of lymphoid follicles in lymph nodes and other lymphatic tissues (e.g., the spleen). MCL often begins as a slow-growing (indolent) disease but tends to become more aggressive over time.
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There are two main types of MCL. The most common MCL subtype is referred to as nodal MCL. This lymphoma usually affects lymph nodes but often spreads to other parts of the body, such as the bone marrow, the bloodstream, the bowel, and the liver. In most patients, nodal MCL is fast-growing although it can also follow a less aggressive (indolent) disease course. Leukemic MCL is less common than the nodal variant and typically results in a swollen spleen. This type of MCL tends to grow slowly and usually does not cause swollen lymph nodes.
MCL is much more common in men than in women and usually affects people who are middle-aged or older. MCL is characterized by a particular mutation in the gene that produces cyclin D1, a protein with an important role in the cell division. As a result of this mutation, leukemic B-lymphocytes in MCL grow out of control.
For the moment, MCL is curable in about 40% of fit patients who can receive intensive treatment. However, the MCL will ultimately return (relapse) in most patients.
Symptoms
The most common symptom of MCL is a painless swelling of lymph nodes. These swollen lymph nodes can develop in any area of the body, but most commonly occur in the neck, armpit, or groin. In addition to this, most MCL patients also have a swollen spleen, resulting in a feeling of fullness after eating and discomfort, or pain behind the ribs. Some patients with MCL may also experience so-called ‘B-symptoms’. This refers to heavy sweating during the night, fever, and an unexplained weight loss over a short period of time.
In most MCL patients, the lymphoma also affects the bone marrow, resulting in a disturbed blood cell production. This may lead to shortness of breath and fatigue, an increased risk of infections and a tendency to bleed or bruise easily.
MCL may also manifest itself outside of the lymph nodes (‘extranodal’ lymphoma). Depending on the location of these extranodal manifestations, patients can experience a variety of symptoms. For example, MCL often grows in the abdomen and bowel, which can lead to abdominal pain, back pain, or diarrhea. In rare cases, the MCL can spread to the brain and spinal cord, which can cause headaches, dizziness, and confusion.
Diagnostic tests
The diagnostic process for MCL starts with a physical examination, during which the doctor palpates lymph nodes and other areas of the body (e.g., the spleen) to check for swelling. The doctor will also assess the medical history of the patient to better understand the general health status and any potential risk factors. However, a definite MCL diagnosis requires a biopsy. This is a small procedure or operation to remove a tissue sample, such as a swollen lymph node, a sample of the spleen, or a bone marrow sample. This sample is sent to a laboratory, where a pathologist assesses whether the specimen contains lymphoma cells and performs additional tests to determine the exact type of cancer cells. In patients with leukemic MCL, the lymphoma can also be diagnosed through a blood test. In most cases of MCL, there are characteristic proteins in the blood (e.g., lactate dehydrogenase) and genetic features (e.g., CDK1 mutations) that can help in the diagnostic work-up.
If these analyses are indicative for MCL, additional tests can be performed to assess how far the lymphoma has spread. This includes a blood test, a bone marrow biopsy to evaluate whether the MCL is also present in the bone marrow and imaging tests (e.g., PET-CT, MRI) to look for signs of MCL in other areas of the body.
When there is a suspicion for central nervous system involvement, patients may also undergo a lumbar puncture (a procedure to take a sample of fluid from your spine) and an MRI scan. Finally, an endoscopy (a camera examination of your stomach or bowel) can be performed when the lymphoma is believed to involve the digestive tract.
Treatments
The treatment strategy for patients with MCL depends on the characteristics of the disease and whether or not the patient experiences symptoms. In patients with a slow-growing form of MCL, who don’t have lymphoma symptoms, physicians often opt for a watch-and-wait strategy. This means that no treatment is given, but that the patient is followed up closely to assess how the MCL evolves.
In case of a fast-growing MCL, or in patients with an indolent form who develop symptoms, treatment is initiated. In patients with localized disease, radiotherapy to the affected areas can lead to long-lasting remissions. For patients with more widespread disease, the preferred first line therapy consists of a combination of chemotherapy with the monoclonal antibody rituximab (chemo-immunotherapy). If this treatment leads to a remission, patients may continue therapy with rituximab alone (maintenance therapy) to further delay a disease relapse.
For fit patients, a stem cell transplantation, followed by maintenance therapy with rituximab is also an option. The most frequent treatment for patients with a disease relapse and for patients who do not respond to the initial therapy (refractory disease) consists of targeted therapy with ibrutinib. Other options for these patients include an alternative chemotherapy regimen, or CAR-T cell therapy (a form of immunotherapy). In addition to this, participating in a clinical trial may offer the opportunity to be treated with new, experimental therapies.
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