Mantle cell lymphoma (MCL) is a rare form of cancer that affects the lymphatic system. It is one of the most aggressive types of lymphoma, with relatively short responses to therapy. People with MCL also experience frequent relapses, even with early and intensive treatment.

Lymphomas are cancers that stem from a type of white blood cells called lymphocytes. MCL occurs due to malignant changes in lymphocytes in the lymph node’s mantle zone, which is the outer edge of a lymph node’s follicle.

The two main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. MCL is a type of non-Hodgkin lymphoma.

This article discusses MCL, including its causes, risk factors, and symptoms. It also looks at diagnosis, treatment, and outlook.

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MCL forms in the lymph nodes, which is why it can quickly spread to other lymph nodes throughout the body. It can also spread to the spleen, bone marrow, blood, liver, and gastrointestinal tract.

Individuals with MCL may experience the following symptoms:

Experts do not know the exact cause of MCL. However, approximately 85% of people with the condition have a genetic change, or mutation, in chromosomes 11 and 14. Short segments of these chromosomes may exchange places.

The median age at diagnosis for people with MCL is 68 years. MCL is also two to three times more common in men than it is in women.

Moreover, in the United States, non-Hispanic white people have the highest incidence of MCL.

To diagnose lymphoma, a doctor will take a detailed patient history, perform a thorough clinical evaluation, and use various specialized tests.

One of these tests is a biopsy of the affected tissue, usually a lymph node, where a doctor takes a sample of the tissue to analyze in a laboratory.

Once the doctor establishes that a person has lymphoma, they will perform a more in-depth examination to determine whether it is MCL. Identifying the correct subtype of lymphoma is essential to ensure a person receives the most suitable treatment.

A doctor diagnoses MCL if they can determine that the lymphoma cells:

  • have surface markers of B cells, which are a type of lymphocyte
  • create excess amounts of a protein called cyclin D1, which stimulates growth
  • have changes in chromosomes 11 and 14

The doctor then performs staging tests to find MCL’s location and how far it has spread.

Determining the stage of the condition is important for developing a prognosis and creating a treatment plan suited to an individual.

The following are tests doctors use to determine the stage of MCL:

  • Complete blood count: This test helps examine the concentration of red blood cells, white blood cells, and platelets.
  • Bone marrow biopsy: This procedure helps determine whether MCL has extended beyond the lymph nodes and into the bone marrow.
  • CT and PET scans: Doctors use these to check whether the condition is affecting the deep lymph nodes, liver, spleen, or other parts of the body.
  • Measurements of lactate dehydrogenase (LDH) and beta-2-microglobulin: These indirect markers of MCL show the extent and rate of progression.

Doctors may use the MCL International Prognostic Index (MIPI) to plan treatment once they know the stage of the condition. The MIPI score depends on a person’s:

  • age
  • ability to perform daily activities
  • LDH levels
  • leukocyte, or white blood cell, count

How well a person can perform daily activities is indicative of how well they could tolerate chemotherapy. The LDH level and leukocyte count indirectly measure disease activity.

The number of factors present at the time of diagnosis determines to which of the three categories — low, intermediate, or high risk — a doctor assigns a person. They then use the assigned category to select the best treatment option.

Chemotherapy is the primary treatment for MCL. Sometimes, doctors combine it with targeted therapy. When possible, chemotherapy is intense, and if a person responds to it well, a stem cell transplant is an option.

If an individual has stage 1 or 2 MCL at diagnosis, doctors often treat it with chemotherapy and immunotherapy, which uses medications to destroy cancer cells. They may also use radiation therapy at the disease site after the patient has undergone chemotherapy, to reduce the cancerous cells further and increase the chances of successful treatment.

If the cancer is in stage 3 or 4 upon diagnosis, a doctor will consider a person’s characteristics, preferences, and clinical and disease factors when choosing a treatment plan.

People who are fit and younger than 65 years old may receive an intensive chemotherapy course with therapy to send the cancer into remission. Autologous stem cell transplant and immunotherapy maintenance can then follow.

Single drug treatments are an option for older adults and those whose bodies are not healthy enough to handle the side effects of combination chemotherapy.

Ongoing treatment can help people live with MCL for many years. Individuals should attend all medical appointments to manage any side effects of medications and allow doctors the chance to spot signs that MCL is returning.

People should ask a doctor about ways to cultivate optimal health. Eating a balanced diet and getting regular exercise are essential for overall well-being.

Individuals may also find that having emotional support from friends, family, or community groups can help manage the emotional difficulties of living with MCL.

According to the Leukemia & Lymphoma Society (LLS), over the past decade, the number of people who survive MCL has nearly doubled, although relapses still commonly occur.

While most patients respond well to initial chemotherapy, MCL remains incurable. For many people, the condition eventually progresses or returns.

MCL does not grow as rapidly as some other fast-growing lymphomas. However, it often does not respond well to treatment. Patients may experience treatment resistance, which can result in less responsiveness to chemotherapy.

People with MCL may also develop an autoimmune disease, which may negatively impact their chance of survival.

According to the LLS, the average amount of time that people with MCL are progression-free is 20 months. The average life expectancy is 5–7 years.

MCL is a type of aggressive cancer that doctors may find challenging to treat.

As new treatments emerge, survival rates improve. Currently, individuals with an MCL diagnosis have an average overall survival time of 5–7 years. Most people have a progression-free period of 20 months.

The survival rate for this cancer has almost doubled over recent decades. In the future, research is likely to further progress, offering more people with MCL an improved outlook.