Marginal zone lymphoma diagnosis often begins with a physical exam that checks for swollen lymph nodes in the neck, underarms and groin and an enlarged spleen. Other tests and procedures include blood tests, imaging tests, endoscopy and taking a sample of tissue for lab testing.

Blood tests

Blood tests can sometimes show whether lymphoma cells are present . Blood tests also can check for infections that may increase the risk of marginal zone lymphoma, such as hepatitis C.

Imaging tests

Imaging tests make pictures of the body. They can show the location and extent of marginal zone lymphoma. Tests might include ultrasound, MRI, CT and positron emission tomography (PET) scans.

Lymph node biopsy

Your healthcare professional may suggest a lymph node biopsy to look for cancer cells. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A lymph node biopsy involves removing all or part of a lymph node. In the lab, tests may show whether you have marginal zone lymphoma.

Bone marrow aspiration and biopsy

Bone marrow aspiration and biopsy are procedures to collect cells from the bone marrow for testing. Bone marrow is the soft matter inside bones where blood cells are made. Bone marrow has a solid part and a liquid part.

In a bone marrow aspiration, a needle is used to draw a sample of the fluid. In a bone marrow biopsy, a needle is used to collect a small amount of the solid tissue. The samples are typically taken from the hip bone. The samples go to a lab for testing. In marginal zone lymphoma, this procedure is done to help determine the cancer's extent.

Endoscopy

An upper endoscopy is a procedure to look at the inside of the esophagus, stomach and first part of the small intestine. A thin, tubelike instrument with a light and a lens for viewing, called an endoscope, is inserted through the mouth and passed down the throat. Tools are passed through the tube to remove a sample of tissue for testing. It may be used if your healthcare professional thinks you have gastric extranodal marginal zone lymphoma. It also may be used to test for H. pylori, which can cause gastric ENMZL.

Testing lymphoma cells in the lab

Lymphoma cells collected from an endoscopy, lymph node biopsy, or bone marrow aspiration and biopsy go to a lab for testing. In the lab, specialized tests look for specific things about the cells. The healthcare team uses the results to find out the type of lymphoma that you have.

To decide whether the cells are marginal zone lymphoma cells, the healthcare professionals in the lab look for:

• Proteins on the surface of the cancer cells. Marginal zone lymphoma cells can be identified by the presence of certain proteins on their surfaces. These proteins are known as markers. Markers can help tell the type of marginal zone lymphoma.
• Changes in the cancer cells' DNA. Cancer happens when cells get changes in their DNA. A cell's DNA holds the instructions that tell a cell what to do. Some marginal zone lymphomas have a gene change that causes their DNA to rearrange. This is known as translocation. This gene change causes the cells to multiply rapidly.

Marginal zone lymphoma staging

If you're diagnosed with marginal zone lymphoma, the next step is to determine the extent of the cancer, called the stage. Imaging tests and biopsies may be used to determine the stage of your marginal zone lymphoma. Your healthcare team uses the cancer stage to help create your treatment plan.

The stages of nodal marginal zone lymphoma range from 1 to 4:

• Stage 1 nodal marginal zone lymphoma. At this stage, only one lymph node or one group of lymph nodes are affected.
• Stage 2 nodal marginal zone lymphoma. Stage 2 involves two or more lymph node regions on the same side of the diaphragm.
• Stage 3 nodal marginal zone lymphoma. At stage 3, lymph node regions on both sides of the diaphragm or lymph nodes above the diaphragm and the spleen are affected.
• Stage 4 nodal marginal zone lymphoma. At stage 4, marginal zone lymphoma has spread outside of the lymph nodes to organs including the bone marrow, liver and lungs.

The stages of extranodal marginal zone lymphoma (EMZL) depend on the location. For gastric EMZL, the stages include 1, 2 and 4:

• Stage 1 gastric EMZL. At this stage, the cancer is confined to the gastrointestinal tract.
• Stage 2 gastric EMZL. Stage 2 disease has extended into the abdomen and nearby lymph nodes.
• Stage 4 gastric EMZL. At stage 4, gastric EMZL is found in multiple sites outside of the lymph nodes or has spread to other organs.

Splenic marginal zone lymphoma (SMZL) isn't staged using 1 to 4. Instead, healthcare professionals look at which body parts are affected to decide the stage.

Marginal zone lymphoma prognosis

The cancer prognosis tells you how likely it is that the cancer can be controlled. Your healthcare team can get a general sense of your outlook using your marginal zone lymphoma stage. But the stage can't tell your future. Your personal prognosis may depend on:

• Your age.
• Your overall health.
• Your LDH level.
• The cancer's stage.

Talk with your healthcare team about your prognosis if you want to know what to expect. Members of your healthcare team can explain what they consider when thinking about your prognosis.

Marginal zone lymphoma survival rates

The chance of surviving marginal zone lymphoma (MZL) is quite good for most people. To understand MZL survival rates, experts study many people with MZL to see how many are living 10 years after the diagnosis. Survival rates vary depending on the type of MZL you have and which treatments you get.

For extranodal marginal zone lymphoma (EMZL) in the stomach caused by H. pylori, the 10-year survival rate when H. pylori is treated is 95%. For EMZL in the stomach not caused by H. pylori, the survival rate is 79%. For EMZLs in other areas of the body, the 10-year survival rate is 89%. The 10-year survival rate for splenic MZL is 53% and for nodal MZL, 72%.

Keep in mind that survival statistics take 10 years to collect. The most recent survival rates include people who had MZL treatment more than 10 years ago. These people may not have had access to the latest treatments.

Marginal zone lymphoma treatments may include treatment for infections, radiation therapy, immunotherapy, chemotherapy, surgery, targeted therapy, CAR-T cell therapy and bone marrow transplant, also called bone marrow stem cell transplant. Which treatment is best for you depends on the extent of your cancer, called the stage. Your healthcare team also considers how quickly the cancer is growing, your overall health and what you prefer.

Treatment for infections

If you have extranodal marginal zone lymphoma in the stomach that's caused by H. pylori, you may be given medicines to treat the infection. Medicines may include antibiotics and proton pump inhibitors. Treating H. pylori can make the cancer shrink or go away in most people.

Antiviral medicines may be given to people with lymphoma caused by hepatitis C to treat the infection. Treating hepatitis C can make the cancer shrink or go away.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation therapy may be used as a first line treatment for extranodal MZL and early-stage nodal MZL. Radiation therapy also may be used to ease symptoms and improve quality of life for people with advanced nodal MZL or splenic MZL that has come back after treatment.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Immunotherapy may be used as a first line treatment for marginal zone lymphoma. It is usually combined with chemotherapy. Immunotherapy also may be used for advanced cancer that has come back after treatment, known as relapsed marginal zone lymphoma, or when other treatments haven't worked, known as refractory marginal zone lymphoma. Immunotherapy may be used alone for older people or people who may not tolerate other treatments well.

Chemotherapy

Chemotherapy treats cancer with strong medicines. There are many chemotherapy medicines. Most chemotherapy medicines are given through a vein. Some come in pill form.

Chemotherapy may be used as a first line treatment for marginal zone lymphoma. It is usually combined with immunotherapy. Chemotherapy also may be used for advanced cancer or cancer that has come back after treatment, known as relapsed marginal zone lymphoma. Chemotherapy may be used after radiation therapy for people with extranodal MZL when other treatments haven't worked, known as refractory extranodal MZL.

Surgery

For splenic marginal zone lymphoma (SMZL) that causes severe symptoms, surgery to remove the spleen, called a splenectomy, may be done. It also may be an option for SMZL that turns into a more aggressive type of lymphoma. SMZL that doesn't cause symptoms may not need treatment right away.

Surgery may be an option for early-stage cancers outside of the lymph nodes such as in the lung, breast, thyroid, and colon or small bowel. Surgery also may be used if the cancer is causing life-threatening side effects such as a blockage or hemorrhage.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die.

For marginal zone lymphoma, targeted therapy may be used as a first line treatment. It is often combined with immunotherapy. Targeted therapy may be used when cancer comes back after treatment, known as relapsed marginal zone lymphoma. Targeted therapy also may be an option when other treatments haven't worked. This is known as refractory marginal zone lymphoma.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. These cells replace cells hurt by chemotherapy and other treatments.

Sometimes, a bone marrow transplant can be used for marginal zone lymphoma that has come back after treatment, called relapsed marginal zone lymphoma.

Monitoring after treatment

Marginal zone lymphoma has a risk of coming back after treatment, known as a relapse. After treatment, you may have follow-up appointments to monitor for relapse. Depending on your type of MZL, you may need follow-up endoscopies, biopsies, blood tests and imaging tests.

Side effects of treatment

Treatments for marginal zone lymphoma may have side effects. These may include nausea, vomiting, fatigue, fever, rash, diarrhea and more. Harmful side effects from treatments may include:

• Bone marrow suppression. When the bone marrow is suppressed, it can't produce enough blood cells, including white blood cells. White blood cells help fight infections, so if you have a lower amount, you are at a higher risk of infections.
• Febrile neutropenia. Febrile neutropenia is a serious condition that can happen in people being treated for cancer. Febrile means having a fever. Neutropenia means having a low number of neutrophils, which are a type of white blood cell that helps fight infections. In febrile neutropenia, the body is trying to fight an infection but doesn't have enough neutrophils.
• Medicine toxicity. Some medicines used to treat cancer can be harmful to the body. They can cause organ damage and other issues. Whether a medicine is toxic depends on the type and how much you take. There are many different medicine options, so your healthcare team can work with you to find the best medicines for you.
• Tumor lysis syndrome. Tumor lysis syndrome is a serious condition that can happen when cancer cells break down quickly after treatment. When these cells die, they release substances into the bloodstream that can overwhelm the body. This can lead to issues with the kidneys and other organs. Medicines may be given to treat or prevent harmful side effects.

With time, you'll find what helps you cope with the uncertainty and worry of a cancer diagnosis. Until then, you may find that it helps to:

Learn enough about marginal zone lymphoma to make decisions about your care

Ask your healthcare team about your cancer, including your test results, treatment options and, if you want, your prognosis. As you learn more about marginal zone lymphoma, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with marginal zone lymphoma. Friends and family can provide the practical support you may need, such as helping take care of your home if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.

Find someone to talk with

Find someone who is willing to listen to you talk about your hopes and worries. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area. Other sources of information include the National Cancer Institute, the American Cancer Society, the Leukemia & Lymphoma Society and the Lymphoma Research Foundation.

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks you might have marginal zone lymphoma, you may be referred to a doctor who specializes in diseases that affect blood cells, called a hematologist. If a cancer diagnosis is made, you also may be referred to a doctor who specializes in treating cancer, called an oncologist.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. It can be hard to remember all the information you get during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For marginal zone lymphoma, some basic questions to ask include:

• Do I have marginal zone lymphoma?
• What type of marginal zone lymphoma do I have?
• What is the stage of my marginal zone lymphoma?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment control my symptoms or prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Do your symptoms happen all the time or do you have them now and then?
• How bad are your symptoms?
• What, if anything, seems to make symptoms better?
• What, if anything, seems to worsen your symptoms?
• Have you had any fevers, night sweats or weight loss?
• Have you noticed any lumps, swelling or pain anywhere in your body?
• Have you had any chest pain, coughing or trouble breathing?
• Have you had any bloating, belly pain or fullness after eating a small amount?
• Have you had any recent infections?
• Do you have any other health conditions, such as diabetes, heart disease or kidney disease?
• What are your biggest worries about your diagnosis or treatment?