Lymphoma is a blood cancer that develops in lymphocytes, a type of white blood cell. Lymphocytes play an important role in your immune system. When they become cancerous, they multiply uncontrollably and grow into tumors.

There are multiple types of lymphoma. The treatment options and outlook vary from one type to another. Take a moment to learn how mantle cell lymphoma (MCL) compares to other types of this disease.

There are two main kinds of lymphoma: Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. There are more than 60 subtypes of non-Hodgkin’s lymphoma. MCL is one of them.

There are two main types of lymphocytes: T lymphocytes (T cells) and B lymphocytes (B cells). MCL affects B cells.

According to the American Cancer Society, Hodgkin’s lymphoma most often affects young adults, especially people in their 20s. By comparison, MCL and other types of non-Hodgkin’s lymphoma are more common in older adults. The Lymphoma Research Foundation reports that most people with MCL are men over the age of 60.

Overall, lymphoma is one of the most common types of cancer to affect children and teenagers. But unlike some types of lymphoma, MCL is very rare in young people.

MCL is much less common than some types of lymphoma. It accounts for roughly 5 percent of all lymphoma cases, according to the American Cancer Society. This means MCL represents about 1 in 20 lymphomas.

Comparatively, the most common type of non-Hodgkin’s lymphoma is diffuse large B-cell lymphoma, which accounts for roughly 1 in 3 lymphomas.

Because it’s relatively rare, many doctors might be unfamiliar with the latest research and treatment approaches for MCL. When possible, it’s best to visit an oncologist that specializes in lymphoma or MCL.

MCL gets its name from the fact that it forms in the mantle zone of a lymph node. The mantle zone is a ring of lymphocytes that surrounds the center of a lymph node.

By the time it’s diagnosed, MCL has often spread to other lymph nodes, as well as other tissues and organs. For example, it may spread to your bone marrow, spleen, and bowel. In rare cases, it may affect your brain and spinal cord.

Swollen lymph nodes are the most common symptom of MCL and other types of lymphoma. If your doctor suspects that you have lymphoma, they’ll take a tissue sample from a swollen lymph node or other parts of your body to examine.

Under a microscope, MCL cells look similar to some other types of lymphoma. But in most cases, the cells have genetic markers that can help your doctor learn what type of lymphoma they are. In order to make a diagnosis, your doctor will order tests to check for specific genetic markers and proteins.

Your doctor might also order other tests, such as a CT scan, to learn if the cancer has spread. They may also order a biopsy of your bone marrow, bowel, or other tissues.

Some types of non-Hodgkin’s lymphoma are low-grade or indolent. That means they tend to grow slowly, but in most cases they’re incurable. Treatment can help shrink the cancer, but low-grade lymphoma usually relapses, or comes back.

Other types of non-Hodgkin’s lymphoma are high-grade or aggressive. They tend to grow quickly, but they’re often curable. When the initial treatment is successful, high-grade lymphoma doesn’t usually relapse.

MCL is unusual in that it shows features of both high-grade and low-grade lymphomas. Like other high-grade lymphomas, it often develops quickly. But like low-grade lymphomas, it’s typically incurable. Most people with MCL go into remission after their initial treatment, but the cancer almost always relapses within a few years.

Like other types of lymphoma, MCL can potentially be treated with one or more of the following approaches:

  • watchful waiting
  • chemotherapy drugs
  • monoclonal antibodies
  • combination chemotherapy and
    antibody treatment called chemoimmunotherapy
  • radiation therapy
  • stem cell transplant

The Food and Drug Administration (FDA) has also approved four medications that specifically target MCL:

  • bortezomib (Velcade)
  • lenalidomide (Revlimid)
  • ibrutinib (Imbruvica)
  • acalabrutinib (Calquence)

All of these medications have been approved for use during relapse, after other treatments have already been tried. Bortezomib has also been approved as a first-line treatment, which can be used before other approaches. Multiple clinical trials are underway to study the use of lenalidomide, ibrutinib, and acalabrutinib as first-line treatments, too.

To learn more about your treatment options, talk to your doctor. Their recommended treatment plan will depend on your age and overall health, as well as where and how the cancer is developing in your body.

MCL is relatively rare and challenging to treat. But in recent years, new therapies have been developed and approved to target this type of cancer. These new therapies have significantly extended the lives of people who have MCL.

If possible, it’s best to visit a cancer specialist that has experience treating lymphoma, including MCL. This specialist can help you understand and weigh your treatment options.