Lymphoma is a cancer that starts in the lymphatic system. The lymphatic system is a network of tissues and organs that remove waste and toxins from the body. Lymphoma includes Hodgkin’s and non-Hodgkin’s lymphoma. This cancer begins in the lymphocytes, which are infection-fighting white blood cells. B-cells and T-cells are two types of lymphocytes that can develop into lymphoma.

Marginal zone lymphoma (MZL) is classified as a group of slow-growing, non-Hodgkin’s B-cell lymphomas.

There are three types of MZL:

1. Extranodal marginal zone B-cell lymphoma or mucosa-associated lymphoid tissue (MALT)

MALT is the most common form of MZL. It can develop in the stomach (gastric) or outside the stomach (non-gastric). It can affect different parts of the body, such as the:

  • lungs
  • small intestines
  • thyroid
  • salivary glands
  • eyes

According to the Lymphoma Research Foundation, this type accounts for 9 percent of B-cell lymphomas.

2. Nodal marginal zone B-cell lymphoma

This rare type develops in the lymph nodes. It accounts for less than 2 percent of all MZL, according to the Lymphoma Association.

3. Splenic marginal zone B-cell lymphoma

This is the rarest form of the disease. It develops in the spleen, bone marrow, or both. According to the American Society of Hematology’s journal Blood, it’s present in less than 2 percent all lymphomas, and has been linked to the hepatitis C virus.

Symptoms of MZL vary, depending on the type. Symptoms associated with all forms of the disease include:

  • fever without an infection
  • night sweats
  • unexplained weight loss
  • skin rash
  • chest or abdominal pain
  • tiredness

You may also have symptoms specific to the type of lymphoma. For example, people with MALT may experience:

  • indigestion
  • stomach pain
  • nausea
  • vomiting

Nodal MZL can cause a painless lump in the groin, armpit, or neck area.

Splenic MZL can cause an abnormal blood count, fatigue, and discomfort due to an enlarged spleen.

The exact cause of nodal and splenic MZL is unknown. In the case of MALT, inflammation due to an infection may be responsible. The disease can develop if you’ve been infected with H. pylori. This bacterium can enter your body and attack your stomach lining.

Although it’s sometimes linked to an infection, MZL isn’t contagious. It’s also not inherited. Certain factors, however, can increase the risk of developing these types of lymphoma. Risk factors include:

  • being 65 years old or older
  • history of a weakened immune system

Treatment can help achieve remission. This is a period when symptoms disappear. Options include:

Treatment depends on the type of MZL and your stage.

1. Gastric and non-gastric MALT

Because MALT is linked to an infection, your doctor may recommend antibiotic therapy over a two-week period. The Lymphoma Research Foundation states that around 70 to 90 percent of people with MALT respond well to this treatment. They also don’t require more treatment.

If lymphoma returns, you’ll also receive traditional cancer therapy in the affected areas. This may include surgery, radiation, or chemotherapy. Your doctor may also prescribe a corticosteroid in combination with cancer treatment. This drug suppresses your immune system and controls inflammation.

2. Nodal MZL

This is a slow-growing form of the disease. If you’re asymptomatic, your doctor may take a watchful waiting approach. This delays treatment until symptoms become apparent. As a result, you avoid grueling side effects of cancer treatment, like anemia, hair loss, fatigue, and nausea. Once symptoms develop, treatment options include chemotherapy, radiation, or surgery.

3. Splenic MZL

Your doctor may suggest surgery to remove an enlarged spleen. This procedure alone may control symptoms. If not, other options include radiation and chemotherapy.

To make a diagnosis, your doctor will need to stage the disease. Staging is also how your doctor decides the right treatment. It involves evaluating the location and size of the tumors and determining whether cancer has spread to other parts of the body.

Your doctor will use imaging tests to capture pictures of inside your body to stage MZL. These imaging tests include X-rays, ultrasounds, CT scans, and MRI scans.

The four staging systems include:

  • Stage 1. MZL is limited to one lymphatic area.
  • Stage 2. MZL is located in more than one lymph node, either below or above the diaphragm.
  • Stage 3. MZL is located in several lymph nodes above and below the diaphragm.
  • Stage 4. MZL has spread to other organs.

Stage 3 and 4 are advanced stages of the disease.

One study found the five-year survival rate to be higher in people with MALT compared to people with splenic and nodal MZL. The study lists five-year survival rates as the following:

  • 88.7 percent for MALT
  • 79.7 percent for splenic MZL
  • 76.5 percent for nodal MZL

Age, stage of the disease at diagnosis, and location affect the outlook for remission and long-term survival. Talk to your doctor if you suspect you might have MZL, and you can work to treat it together. With early diagnosis and treatment, remission is possible and the outlook is positive.