If You Have Waldenstrom Macroglobulinemia

If you or someone you know has been diagnosed with Waldenstrom macroglobulinemia (WM), this guide can help you understand what it is and how it is treated.

What is Waldenstrom macroglobulinemia?

Cancer starts when cells in your body begin to grow out of control. Cells in nearly any part of your body can become cancer. Waldenstrom macroglobulinemia (WM) is a type of non-Hodgkin lymphoma, a cancer that starts in a kind of white blood cell called lymphocytes.

The cancer cells in WM make large amounts of an abnormal protein called a macroglobulin. Each protein made by the WM cells is the same, so it is known as a monoclonal protein, or just an M protein.

Many of the symptoms of WM happen when this protein builds up in your body. This includes symptoms like too much bleeding, problems with vision, and nervous system problems.

WM cells grow mainly in your bone marrow, where they can crowd out normal cells. This can lead to:

• Low levels of red blood cells (anemia), which can make you feel tired and weak
• Low levels of white blood cells, which makes it hard for your body to fight infection
• Low levels of platelets, which can lead to increased bleeding and bruising

These cancer cells can also grow in lymph nodes and organs like the liver and spleen. When this happens, these organs can swell, causing abdominal pain and fullness.

Questions to ask the doctor:

• Why do you think I have cancer?
• Is there a chance I don’t have cancer?
• Would you please write down the kind of cancer I have?
• What will happen next?

How does the doctor know I have Waldenstrom macroglobulinemia?

Waldenstrom macroglobulinemia (WM) is often found when a person goes to see their doctor because they are having symptoms or because they just don’t feel well and go in for a checkup.

Sometimes, it is found in people without symptoms when they have blood tests for some other reason.

Common symptoms of WM include:

• Weakness
• Loss of appetite
• Fever
•  Sweats
• Weight loss
• Neuropathy (numbness or a pins-and-needles feeling in the feet/legs)

If you have symptoms that could be from WM, your health care team will do exams and tests to be sure. They will want to get a complete medical history to learn more about the symptoms. They will also do a physical exam and may do other tests as well.

The most important tests will look for abnormal proteins in your blood and abnormal cells in your bone marrow.

Tests that may be done

Blood cell counts: This blood test is often the first test done. People with WM may have a low red blood cell count.

Blood tests for immunoglobulins: Samples of your blood might be tested for immunoglobulins (another name for antibodies). Levels of one of these proteins (IgM) are typically higher than normal in people with WM.

Viscosity: Viscosity is a measure of how thick your blood is. If the IgM protein level is too high, your blood will become thick, or viscous, and cannot flow freely (think about pouring honey compared to pouring water).

Bone marrow aspiration and biopsy: For these tests, a doctor uses thin, hollow needles to remove small amounts of bone marrow, usually from your hip bone. The area around the bone is numbed and you may be given a drug to help you relax during the test. The samples are sent to a lab to see if there are WM cells in your bone marrow.

Other biopsy tests: If you have a tumor or an enlarged lymph node, a thin, hollow needle attached to a syringe might be used to remove a sample of it. Sometimes the sample is removed with surgery instead. The sample is then tested for cancer cells.

CT scan: This test uses x-rays to make detailed pictures of the inside of your body. It can be used to see the extent (spread) of the cancer and is sometimes used to plan a biopsy.

MRI scan: MRIs make detailed pictures using radio waves and strong magnets instead of x-rays. MRIs make it easier to see the extent (spread) of the cancer.

PET scan: PET scans use a special kind of sugar that can be seen inside the body with a special camera. If there is cancer, this sugar shows up on the test. If doctors see something on another test, a PET scan can help them figure out if it is cancer.

Questions to ask the doctor:

• What tests will I need?
• Who will do these tests?
• Where will they be done?
• How and when will I get the results?
• Who will explain the results to me?
• What do I need to do next?

How serious is the cancer?

If you have Waldenstrom macroglobulinemia (WM), your cancer care team will gather some key pieces of information to help decide if you need treatment.

For most types of cancer, doctors define the cancer stage. This describes how much cancer is in the body. However, there is no staging system for WM.

In WM, the presence or absence of symptoms is the most important factor doctor use to decide if someone needs treatment.

• Asymptomatic WM: a person has WM but no symptoms
• Symptomatic WM: a person has WM with symptoms

A few different scoring systems are used to estimate when asymptomatic WM may develop symptoms and how long someone might live with symptomatic disease.

In people with WM but no symptoms, doctors look at factors like levels of certain proteins in the blood (albumin, β2M, IgM) and the amount of cancer cells in the bone marrow to estimate how quickly treatment may be needed in the future.

In people with WM and symptoms, doctors look at factors like a person’s age and levels of certain proteins in the blood (β2M, LDH, albumin) to estimate how long a person may live with WM.

Be sure to ask the cancer care team about the specifics of your cancer and what it means for you.

Questions to ask the doctor:

• Do you know how advanced my cancer is?
• Based on the specifics of my cancer, how long do you think I will live?
• What will happen next?

What kind of treatment will I need?

The treatment plan that is best for you depends on your age, overall health, and other factors, such as symptoms you are having. If the WM is found early and isn’t causing symptoms, you might not need treatment right away. You might just be watched closely instead. If you do need treatment, it could include:

• Chemotherapy
• Targeted therapy
• Immunotherapy
• Stem cell transplant
• Plasmapheresis

Chemotherapy (chemo)

Chemo is the use of drugs to kill cancer cells. These drugs go into your bloodstream and spread to cells all over your body. Chemo is often given in cycles or rounds. This allows for breaks so your body can recover from treatment. Chemo may be given alone or in combination with immunotherapy and/or targeted therapy.

Possible side effects

Chemo can have short-term side effects like:

• Hair loss
• Mouth sores
• Loss of appetite
• Nausea and vomiting
• Diarrhea
• Feeling tired (from low red blood cells, called anemia)
• Bruising or bleeding easily (from low platelets, called thrombocytopenia)
• Increased risk of infection (from low white blood cells, called leukopenia)

There are ways to lessen and treat many chemo side effects. Tell your cancer care team about any side effects so they can help.

There are also long-term side effects of chemo drugs. Your risk of long-term side effects will depend on what drugs are used for your treatment. Talk with your cancer care team to learn more.

Other drug treatments

Many other types of drugs can be used to treat WM or its symptoms. These drugs work differently from chemo.

• Targeted therapy targets parts of WM cells that make them different from normal cells.
• Immunotherapy helps your immune system attack the WM cells.

Different types of drugs are often combined, sometimes along with chemo.

Some drugs for WM are taken as pills, while others are injected into a vein. Each of these drugs can have its own side effects, so ask your cancer care team which drugs you will get and what to expect.

Plasmapheresis

Plasmapheresis (plasma exchange) is a procedure to remove the abnormal proteins made by WM cells. You might have plasmapheresis to make your blood less thick and lower your risk of bleeding problems or stroke.

Stem cell transplant (SCT)

Stem cell transplant (SCT) can be used as a part of treatment for WM.

In an SCT, high doses of chemotherapy are used to kill WM cells. These higher doses of chemo also destroy your bone marrow, keeping your body from making new blood cells. To reverse this side effect, new stem cells are returned to your body after high dose chemo to bring back the normal blood-making bone marrow stem cells.

There are different kinds of SCT, each of which can have side effects. Ask your cancer care team if an SCT is part of your treatment plan, which type you might have, and what to expect.

Clinical trials

Clinical trials are research studies that test new drugs or other treatments in people. They compare standard treatments with others that may be better.

Clinical trials are one way to get the newest cancer treatment. They are also the best way for doctors to find better methods of treating cancer. But they might not be right for everyone. If you do sign up for a clinical trial, you can always stop at any time.

If you would like to know more about clinical trials, start by talking to your cancer care team. You can also find more information in Clinical Trials.

What about other treatments I hear about?

You might hear about other ways to treat cancer or your symptoms. These may not always be standard medical treatments. These treatments may be vitamins, herbs, special diets, and other things.

Some of these might help, but many have not been tested. Some have been shown not to help. A few may be harmful or unsafe when combined with other treatments your cancer care team suggests. Talk to your cancer care team about anything you are thinking of using, whether it is a vitamin, a special diet, or anything else.

Questions to ask the doctor:

• Do I need other tests before we decide on treatment?
• Will any other doctors be on the treatment team? What do they do?
• What treatment do you think is best?
• What is the goal of this treatment? Do you think it could cure my cancer?
• Will I need other types of treatment, too?
• What is the goal of these treatments?
• What side effects could I have from treatment?
• What can I do about them?
• Should we think about a stem cell transplant? If so, when?
• Is there a clinical trial that might be right for me?
• What about special vitamins or diets I hear about? How will I know if they are safe?
• How soon do I need to start treatment?
• What should I do to be ready?
• Is there anything I can do to help the treatment work better?
• What is the next step?

What will happen after treatment?

Treatment often doesn’t cure WM, but it might make it go away for a time. If you have WM, there might be times when you aren’t being treated. Or you might continue regular treatments with chemotherapy and other drugs or treatments to try to keep the cancer in check.

Whether or not you are being treated, ongoing follow up is very important.

During follow-up appointments, your cancer care team will ask you about symptoms and do an exam. They might also order blood tests or other testing. This is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have.