Marginal Zone Lymphoma

Written by Stephanie Booth
Medically Reviewed by Elmer Huerta, MD, MPH on May 15, 2025
13 min read

Marginal zone lymphoma (MZL) isn’t one disease. It’s a group of rare, slow-growing blood cancers. They’re known as non-Hodgkin lymphomas because they start in your lymphatic system.

Your lymphatic system is made up of organs and tissues that help protect you from infection. MZL causes your B cells, special white blood cells found in your lymph nodes and other parts of this system, to change.

While there’s no cure for marginal zone lymphoma, treatments can try to stop it from spreading. Because it grows so slowly, you might not even need treatment right away. That will depend on the type you have.

You might not know you have MZL until your doctor spots signs in a physical exam, blood work, or imaging test (like an ultrasound).

What Is the first sign of marginal zone lymphoma?

Doctors refer to the most common MZL signs as “B symptoms” because your B cells are affected. They are:

  • Night sweats
  • Fever
  • Weight loss without trying

You could also not have any symptoms at all. 

There are three types of MZL. Beside B symptoms, each kind can cause other symptoms.

Splenic marginal zone lymphoma

You may have:

  • Fatigue (feeling tired even after resting)
  • Enlarged spleen (you may feel pain or fullness in the upper left part of your belly)

MALT lymphoma

If you have this type of MZL, you could have:

  • Skin rash
  • Eye discharge (you may mistake it for pink eye)
  • Bloating for no reason
  • Stomach pain
  • Throwing up

Nodal marginal zone lymphoma

With this kind, you may also have:

  • Swollen lymph nodes, usually in your neck
  • Fatigue
  • Anemia (low red blood cells)

Because all types of marginal zone lymphoma grow slowly, you could have it for years before you start having symptoms.

Here’s a closer look at the kinds of MZL and what makes them different.

MALT lymphoma (extranodal marginal zone lymphoma of mucosa-associated lymph tissue)

This is the most common type of MZL. If you have it, it means that lymphocytes (B cells) have started growing out of control in lymphoid tissue outside your lymph nodes. 

Mucosa tissue refers to the moist lining that’s found in different parts of your body, like the inside of your mouth or the lining of your gut.

One-third of the time, MALT lymphoma starts in your stomach. This is called gastric (stomach) MALT lymphoma.

You also have mucosa tissue in places like your skin, salivary glands (which help you make spit), eyes, bowels, and lungs. If B cells start mutating in one of these areas, it’s called non-gastric MALT lymphoma. 

Nodal marginal zone lymphoma

This is the second most common type of MZL. If you have it, abnormal B cells are in your lymph nodes, pea-sized glands that you have throughout your body. Lymph nodes are like tiny waste stations that collect and flush out germs.

Splenic marginal zone lymphoma

This is the rarest type of MZL. It starts in your spleen, an organ that’s an important part of your lymphatic system. It helps filter germs from your blood.

Your genes are at the root of this group of cancers. 

Sometimes mutated genes are passed down to you from your parents. But more often, the gene changes that cause marginal zone lymphoma are triggered by environmental factors (things you’re exposed to) during your life.

Researchers are still trying to find all the different gene changes that could cause MZL. 

Long-term inflammation from an infection also appears to trigger some types.

You can’t catch any type of cancer from someone else. That means that you can safely share a meal, hold hands, and kiss someone with MZL without worrying that you’ll get sick, too.

But some types of infections are known to raise your risk for marginal zone lymphoma, and a few of those can be contagious. 

Anyone can get MZL, but it’s more common if you’re 60 or older. More women are diagnosed than men. Having a lot of infections or living with an autoimmune disease, like Sjogren's syndrome or Hashimoto’s disease, could also raise your risk.

 Some bacterial infections are believed to raise your risk of MALT marginal zone lymphoma. Among them are:

  • Helicobacter pylori (H. pylori). This common stomach bacteria causes ulcers and heartburn. 

  • Chlamydophila psittaci. If you get infected with this bacteria, it may raise your risk of MALT lymphoma that affects your eyes.

  • Borrelia burgdorferi. This is the same germ that is spread by ticks and causes Lyme disease.

  • Campylobacter jejuni. A common strain of food poisoning might help trigger this type of MZL in your small intestine.

 

The first step is often a physical exam, where your doctor may check for swollen lymph nodes in your neck and other parts of your body. They’ll ask about any symptoms you’re having and your family’s health history. 

If your doctor thinks that you could have marginal zone lymphoma, they’ll order tests to get more information.

Tests for marginal zone lymphoma

These can include:

  • Complete blood count (CBC)
  • Blood tests. These can check for infections like hepatitis C, special proteins that cancer cells make, or signs of tissue damage.
  • CT scan
  • Liver function test
  • Kidney function test
  • Bone marrow aspiration. This procedure checks to see if your bone marrow is making cancer cells.
  • Biopsy. A tiny tissue sample can be removed and sent to a lab to look for cancer cells.

What to know when you’re diagnosed

There’s no “right” way to learn that you have cancer. You may feel a range of emotions or wonder, “Why me?” 

You may find it helpful to share how you’re feeling. Talk about your diagnosis with your loved ones. Ask your doctor about an online or in-person support group you could join. You may also find that it feels good to talk to a mental health expert, like a counselor or therapist. They can help you sort through all the feelings you’re having.

How to deal with a sudden diagnosis

It can take time to process the news of your diagnosis. It may help to:

Take things day by day. If your usual tasks feel too hard, break them into bite-sized pieces so they feel easier to manage.

Carve out time to do things you love. Even small moments of joy can help you relax and recharge.

Try not to blame yourself. Your diagnosis is not your fault. Many different factors cause marginal zone lymphoma, and there’s a lot that experts still don’t know about it. If you find it hard to be kind to yourself, ask yourself what you would say to a loved one.

Many different people will help you manage your diagnosis and treatment. They include:

  • Primary care doctor

  • Pharmacist

  • Nutrition specialist

  • Social worker

  • Nurses. For instance, they’ll help prepare you for procedures and monitor you as you recover.  

  • Medical oncologist or hematologist. This cancer specialist can figure out a treatment plan that could include chemotherapy, immunotherapy (which uses your own immune system to fight the cancer) or targeted therapy (special drugs that alter how your immune system works.)

  • Radiation oncologist. When radiation is needed to kill cancer cells, this doctor will guide you through the process.

  • Bone marrow or stem cell transplant specialist. Stem cells are the only cells in your body that can turn into different specialized cells. Sometimes getting new ones can treat certain types of cancer. A transplant specialist can help with this.

  • Surgeon

 

Your treatment will depend on the type of MZL you have, as well as how far it’s already spread. 

Medications for marginal zone lymphoma

These could include:

  • Antibiotics. Because many cases of MZL are associated with bacterial infections, between 30% to 40% of MALT lymphoma cases can be cleared up with antibiotics.

  • Antiviral drugs. You might take these if you have a virus like hepatitis C.

  • Biologics. These drugs are made with living matter, like genetic material. They help alter how your immune system works. 

  • Chemotherapy drugs. If your doctor prescribes chemotherapy (chemo), you’ll get a medication that can destroy the fast-growing cancer cells in your body. You usually get these drugs through an IV (a vein in your arm).

  • BTK inhibitor. Every B cell has a BTK protein. This new kind of drug binds to it, which prevents more cancer cells from growing.

Other therapies for marginal zone lymphoma

  • Stem cell transplant. Your doctor may want you to have this after you get high doses of chemotherapy. 

  • Radiation therapy. This is when high doses of radiation are used to destroy the cancer cells in your body. This doesn’t happen right away, so you’ll likely need more than one treatment session. 

Surgeries for marginal zone lymphoma

If your spleen is very swollen, your doctor may need to remove it. This procedure is called a splenectomy.

Natural treatment for marginal zone lymphoma

If your cancer is in an early stage, your doctor could give you the option of not starting treatment right away. This is called “watchful waiting” or “active surveillance.”

You’ll still see your doctor for regular physical exams and lab work. If the cancer starts to grow or spread, your doctor will then start your treatment.

Watchful waiting can help you put off the side effects and challenges that can happen with cancer treatment. But it doesn’t treat MZL, and it’s not the right choice for everyone. 

Home remedies for marginal zone lymphoma

Many products are sold online as cancer cures, but there’s no proof that they work. In some cases, they could be harmful.

Always talk to your doctor before taking a new herb, supplement, oil, or other product that claims to treat your cancer.

Once you’re diagnosed, your doctor will stage your cancer. This is a way to describe it based on how far it’s spread. This may be done with a Roman numeral (like I, II, III, or IV) or sometimes with a number (like 1, 2, 3, or 4.) 

Staging helps your doctor choose a treatment plan. It can also help you better understand your diagnosis.

Stage I

This is the earliest stage. It means that your cancer was found early. Cancer cells are only in one area of lymphatic tissue. 

Stage II

If you’re diagnosed with stage II marginal zone lymphoma, cancer cells are in at least two lymph nodes. They are above or below your diaphragm, the muscle that separates your chest from your belly.

Stage III

Cancer cells are in III or more lymph nodes both above and below your diaphragm.

Stage IV

In this advanced stage, cancer cells have spread to more than one of your organs.

Treatments for MZL could cause: 

  • A weak immune system. It will be very easy for you to get sick, so you’ll need to take extra steps to stay safe from germs. 

  • Infertility. Both chemotherapy and radiation can make it harder for you to get pregnant or carry your baby to term. Sometimes this is only a short-term issue. If you want to start a family in the future, your doctor may suggest that you freeze your eggs or store sperm before treatment starts.

Other health issues

Going through treatment for marginal zone lymphoma can also raise your risk for: 

  • Other types of cancer
  • Lung disease
  • Heart disease
  • Depression

You and your doctor can work together to try to avoid or manage these issues.

A cancer diagnosis may make you feel helpless. But there’s a lot that you can do to support yourself.

Marginal zone lymphoma diet

Eating healthy foods can help reduce inflammation inside your body and improve how well your immune system works. It may also help you manage side effects during cancer treatment. Try to focus on:

  • Fruits and vegetables, which are high in vitamins and minerals
  • Plant-based proteins (like beans, legumes, nuts, and seeds)
  • Healthy fats (look for monounsaturated or polyunsaturated fats like you find in avocados and olive oil)
  • Healthy carbohydrates (such as 100% whole wheat bread, bran cereal, and brown rice)

If you need help with meal ideas, a registered dietitian or nutritionist can help.

Marginal zone lymphoma cost

A lot of factors go into the cost of MZL treatment, including whether or not you have health insurance, the doctor you see, the treatment you have, and any complications that come up.

Because of that, it’s hard to predict how much your treatment for marginal zone lymphoma will be. One study found that the average cost is over $9,000.

How Is marginal zone lymphoma managed? 

It’s important for you to work closely with your doctor, especially because MZL can go away and come back. To protect your health:

Keep all your doctor appointments even if you’re not currently in treatment or don’t feel sick.

Take all your medication exactly as prescribed. Don’t start or stop anything without first checking with your doctor.

Don’t “power through” any treatment side effects. Your doctor has lots of ways to help you manage them.

Tell your doctor right away if you get sick. They’ll want to make sure that your symptoms don’t get worse or cause other health issues.

Marginal zone lymphoma and stress

Even if you’re not having symptoms, it can feel stressful to know you have cancer. To take care of your mental health:

Accept how you’re feeling. Identify the emotions you’re having and let yourself have them. Writing in a journal may help.

Try different ways to relax. Mindfulness techniques like yoga and meditation can remind you to be in the present. You might also listen to music you love or curl up with your pet.

Ask questions. Getting more information about your condition may help lessen the uncertainty you feel. Your doctor is the best resource for this.

Get more help if you need it. A therapist can give you a safe place to vent, either in private or group sessions. A social worker can help you work through money, work, or family issues. You might also talk to your doctor about antidepressants or anxiety medication.

Support for marginal zone lymphoma

Aside from your loved ones, many cancer nonprofits can offer support.

For instance, some have free services that can help you deal with health insurance, figure out foods to eat during treatment, and answer questions about a specific treatment. They can also connect you to support groups or clinical trials, which rely on volunteers to try brand new treatments.

A few groups to look into include:

Ask your doctor if they can suggest a local group.

No one can say for sure what your outcome will be. Everyone is different and many factors are involved, from the type of treatment you have to how well it works for you. Your doctor is the best person to explain your outlook.

Can marginal zone lymphoma be cured?

It depends on the type you have. If the cause of your cancer is an H. pylori infection, antibiotics, usually given with some other medications, may be able to cure it in a few months.  

With other types of MZL, treatment can put you in remission. That means that you’re cancer-free and have no symptoms. But there is a chance that your cancer will come back.

What to expect with marginal zone lymphoma?

Five-year survival rates look at other people who had your same type of cancer and estimate how many were alive five years after they were diagnosed. For people with MZL, these are the five-year survival rates:

  • 88% for extranodal marginal zone lymphoma
  • 79% for splenic marginal zone lymphoma
  • 76.5% for nodal marginal zone lymphoma

These numbers are only estimates. Your experience and outcome could be different.

MZL is a complex condition that has to do with your genes and many other things outside of your control. Doctors haven’t found any way to prevent it. 

Marginal zone lymphoma is a group of rare blood cancers. They start in your lymphatic system, which helps your body fight infection. Many treatments can help, and because MZL is so slow-growing, you may not even need to start them right away.

What questions should I ask my doctor? After learning that you have cancer, you might want to ask:

  • What type of MZL do I have?
  • What stage is it in?
  • What treatment do I need?
  • Is “watchful waiting” an option?
  • Will I need time off work?
  • What support services can you connect me with?

When should I tell people I have cancer? Only you can decide when to share your news, who to tell, and which details to include. It may feel scary, but talking about your diagnosis could help you sort through your feelings and plan your next steps.

Think ahead of what you might say, as well as how you’ll set boundaries. For instance, if you’re asked a question that you don’t want to answer, you could say, “Thanks for asking, but I’m not ready to talk about it right now.”

Will my cancer come back after treatment? Twenty percent of people who have marginal zone lymphoma see their cancer return within two years of treatment. But there are many treatment options your doctor can try. Researchers keep learning more about MZL and finding new ways to treat it.