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 can also experience frequent relapses.

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:

  • loss of appetite
  • unexplained weight loss
  • fever
  • night sweats
  • nausea
  • vomiting
  • indigestion
  • abdominal pain
  • bloating
  • pressure or pain in the lower back
  • fatigue

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 60–65 years. MCL is also two to three times more common in people assigned male at birth than people assigned female at birth.

MLC is also more common in non-Hispanic white people in the United States.

To diagnose lymphoma, a doctor will:

  • take a detailed medical history
  • perform a thorough clinical evaluation
  • order various 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.

If a doctor suspects MCL with gastrointestinal involvement, they may recommend tests such as esophagogastroduodenoscopy and colonoscopy.

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.

How do doctors stage mantle cell lymphoma?

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 the 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. Stage I refers to early localized cancer, while stage IV indicates that the condition is widespread.

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

  • Blood tests: These can include complete blood count, comprehensive metabolic panel, uric acid test, and measurements of lactate dehydrogenase (LDH) and beta-2-microglobulin.
  • 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 for mantle cell lymphoma 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 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.

In around 10–15% of cases, doctors recommend watchful waiting. This is when the condition is low risk and does not require treatment straight away.

Possible treatments include:

  • chemotherapy
  • targeted therapy
  • stem cell transplant
  • immunotherapy
  • radiation therapy

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, such as the monoclonal antibody rituximab, to destroy cancer cells.

They may also use radiation therapy at the site of the condition after the individual has undergone chemotherapy. This helps to reduce the cancerous cells further and increase the chances of successful treatment.

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.

While most individuals 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. People 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.

Ongoing treatment can help people with MCL. 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.

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

Here are some frequently asked questions about MCL.

What is the survival rate for mantle cell lymphoma?

According to the Leukemia & Lymphoma Society, the median overall survival is 4–5 years. This means that people typically live for 4–5 years after the initial diagnosis or after beginning treatment.

How curable is mantle cell lymphoma?

It is not possible to cure MCL. However, treatments can help to ease symptoms, improve comfort, and possibly extend a person’s life.

Is mantle cell lymphoma an aggressive cancer?

MCL is an aggressive type of cancer. In most cases, treatment is necessary immediately.

Mantle cell lymphoma (MCL) is a type of aggressive cancer that doctors may find challenging to treat.

MCL can cause symptoms such as nausea, vomiting, indigestion, loss of appetite, and more. Although the condition is incurable, treatments can help manage symptoms.

It is best to contact a doctor as soon as there are concerns about MCL. The doctor can order tests to confirm the diagnosis and advise on a suitable treatment plan.