Waldenstrom macroglobulinemia (mak-roe-glob-u-lih-NEE-me-uh) is a type of cancer that begins in the white blood cells. Waldenstrom macroglobulinemia is considered a type of non-Hodgkin lymphoma. It's sometimes called lymphoplasmacytic lymphoma.

In Waldenstrom macroglobulinemia, some white blood cells undergo changes that turn them into cancer cells. The cancer cells can build up in the spongy material inside the bones where blood cells are made. This material is called bone marrow. The cancer cells crowd healthy blood cells out of the bone marrow. Cancer cells also may build up in other parts of the body, such as the lymph nodes and the spleen.

The cancer cells make a protein called immunoglobulin M (IgM) that can build up in the blood. Too much IgM can reduce blood flow in the body, which can damage the organs and cause other problems.

Waldenstrom macroglobulinemia can't be cured. Treatments aim to ease symptoms and reduce organ damage. Treatment options may include a "watch and wait" approach, plasma exchange, targeted therapy, chemotherapy, immunotherapy and a bone marrow transplant, also known as a bone marrow stem cell transplant.

Waldenstrom macroglobulinemia grows slowly. It might not cause symptoms for years.

When they happen, Waldenstrom macroglobulinemia symptoms may include:

• Fatigue.
• Fever.
• Drenching night sweats.
• Weight loss that happens without trying.
• Numbness or weakness in the arms or legs.
• Swollen lymph nodes, which might feel like a lump under the skin. They happen most often in the neck, armpits and groin.
• A feeling of pain or fullness under the ribs on your left side, which may be caused by an enlarged spleen.
• Easy bruising.

Waldenstrom macroglobulinemia sometimes causes thickened blood and slow blood flow, called hyperviscosity syndrome. It can happen if the cancer cells make too much IgM that builds up in the blood. Hyperviscosity syndrome symptoms include:

• Vision changes such as blurry vision, double vision or vision loss.
• Headache.
• Dizziness.
• Ringing in the ears.
• Sudden hearing loss.
• Bleeding nose or gums.
• Shortness of breath.
• Confusion.
• Memory loss.
• Fainting.

When to see a doctor

Make an appointment with a healthcare professional if you have any symptoms that worry you. If you have symptoms of hyperviscosity syndrome, seek emergency medical attention right away.

It's not clear what causes Waldenstrom macroglobulinemia. Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

In Waldenstrom macroglobulinemia, the changes happen in the white blood cells. The changes turn some of the white blood cells into cancer cells. The cancer cells can build up in the spongy material inside the bones where blood cells are made. This material is called bone marrow. The cancer cells crowd healthy blood cells out of the bone marrow. The cancer cells also may build up in the lymph nodes and the spleen.

Waldenstrom macroglobulinemia cells make a protein that the body can't use called IgM. IgM can build up in the blood. This may reduce blood flow in the body and cause other problems.

Factors that can increase the risk of Waldenstrom macroglobulinemia include:

• Older age. Waldenstrom macroglobulinemia can happen at any age. But it's most common in adults 70 and older.
• Race and ethnicity. White people are more likely to develop Waldenstrom macroglobulinemia than are people of other races.
• A family history of lymphoma. Having a blood relative, such as a parent or sibling, with Waldenstrom macroglobulinemia may increase your risk of Waldenstrom macroglobulinemia.
• A history of autoimmune diseases. People with a history of autoimmune diseases are at a higher risk of Waldenstrom macroglobulinemia.

Complications of Waldenstrom macroglobulinemia may include:

Hyperviscosity syndrome

Some people with Waldenstrom macroglobulinemia develop hyperviscosity syndrome. Hyperviscosity refers to the thickening of the blood that leads to slower flow through the blood vessels. In Waldenstrom macroglobulinemia, this happens because of the increased proteins in the blood. Hyperviscosity syndrome can cause life-threatening complications.

Bing-Neel syndrome

Bing-Neel syndrome describes when Waldenstrom macroglobulinemia involves the central nervous system. The central nervous system includes the brain and spinal cord. Bing-Neel syndrome can cause headaches, seizures, weakness or numbness in the limbs, and unusual nerve pain or tingling. Bing-Neel syndrome can develop when a person is in remission or, less commonly, when the cancer first starts. The outlook is usually worse when it develops later.

A switch to a more aggressive form of cancer

Some people with Waldenstrom macroglobulinemia can develop a more aggressive form of cancer.

Secondary cancers

People with Waldenstrom macroglobulinemia are at a higher risk of developing other cancers. These may include thyroid cancer, kidney cancer and melanoma.

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

Blood tests

Tests and procedures used to diagnose Waldenstrom macroglobulinemia include blood tests that:

• Count the number of cells in a blood sample. A complete blood count can show if there are too few healthy blood cells.
• Detect a protein made by cancer cells. Blood tests can detect whether a protein made by cancer cells is in the blood. In Waldenstrom macroglobulinemia, the protein is called immunoglobulin M (IgM). An increased level in IgM is known as an M spike.
• Show how the organs are functioning. Blood tests can show whether the IgM proteins are harming organs, such as the kidneys and the liver.
• Measure how the blood flows. A serum viscosity test measures the blood's resistance to flow. In Waldenstrom macroglobulinemia, higher amounts of IgM proteins can thicken the blood and make it flow more slowly.
• Measure levels of lactate dehydrogenase. Lactate dehydrogenase (LDH) levels in the blood are often higher in people with lymphoma.
• Check for viruses. Blood tests can check for viruses, including human immunodeficiency virus (HIV), hepatitis B and hepatitis C. The presence of a virus may affect treatment options.

Bone marrow biopsy and aspiration

Bone marrow biopsy and aspiration are procedures that involve collecting cells from the bone marrow. The cells are sent for testing.

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 most often come from the hip bone. The sample goes to a lab where it is tested for cancer cells. If there are cancer cells, more tests can give more information about the cells.

Imaging tests

Imaging tests make pictures of the body. They can help show whether cancer has spread to other areas of the body. Tests might include CT scan and positron emission tomography (PET) scan. If your healthcare professional suspects that Waldenstrom macroglobulinemia involves your brain and spinal cord, a condition known as Bing-Neel syndrome, your healthcare professional may suggest an MRI.

Lumbar puncture

A lumbar puncture is a procedure to collect fluid from around the spinal cord. A lumbar puncture, also called a spinal tap, uses a needle. A healthcare professional inserts the needle between two bones in the lower back and draws out some of the fluid that surrounds the brain and spinal cord. This fluid is called cerebrospinal fluid. The fluid goes to a lab where it's tested for cancer cells.

A healthcare professional might do a lumbar puncture if you have symptoms of Bing-Neel syndrome, such as headaches, seizures, weakness or numbness in the limbs, and unusual nerve pain or tingling. The test looks for cancer cells in the central nervous system, which includes the brain and spinal cord.

Tissue biopsy

If your healthcare professional suspects Bing-Neel syndrome, they may suggest a biopsy of tissue from the central nervous system. A biopsy is a procedure to remove a sample of tissue for testing in a lab. In the lab, tests may show whether you have Bing-Neel syndrome.

Testing cells in the lab

Cells collected from a biopsy or a 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 decide if you have Waldenstrom macroglobulinemia.

To decide whether the cells are Waldenstrom macroglobulinemia cells, the healthcare professionals in the lab look for:

• Proteins on the surface of the cancer cells. Cancer cells have certain proteins on their surfaces that help identify them. These proteins are known as markers. Common markers in Waldenstrom macroglobulinemia include CD19, CD20 and CD22. The lab tests also can show if the IgM protein is present.
• Changes in the cancer cell DNA. Cancer happens when cells get changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In Waldenstrom macroglobulinemia, the changes, which are sometimes called gene mutations, happen in genes called MYD88 and CXCR4. The changes tell the cells to make many more cells, and they let cells keep living when healthy cells would die. The mutations in your cancer cells may help your healthcare team decide if your cancer is likely to grow quickly.

Stages

Waldenstrom macroglobulinemia doesn't have stages. Other cancers use stages, such as 1, 2, 3 or 4, to describe how far the cancer has spread. Waldenstrom macroglobulinemia affects the blood and bone marrow and is often found throughout the body, so the usual stages don't apply. Instead, healthcare professionals use risk groups to describe the seriousness of the disease and to help plan care.

The healthcare team assigns a risk group using the Modified Staging System for Waldenstrom macroglobulinemia. It places people into one of four groups based on their age and results from blood tests.

The risk groups include:

• Low risk.
• Low-intermediate risk.
• Intermediate risk.
• High risk.

People in the lower risk groups tend to live longer and often need less frequent monitoring. Those in the higher risk groups have a higher chance of dying of Waldenstrom macroglobulinemia and may need closer follow-up or earlier treatment.

To decide on the risk group, your healthcare team uses:

• Your age. Being older than 65 increases your risk. Being older than 75 increases your risk even more.
• The levels of LDH in your blood. An LDH level that is higher than usual increases your risk.
• The amount of a specific protein, called albumin, in your blood. A low level of albumin in your blood increases your risk.

The risk groups don't determine when to start treatment. Instead, they help healthcare professionals talk about outlook, guide follow-up and plan long-term care. Many people with Waldenstrom macroglobulinemia live for years with treatment, and new therapies continue to improve survival.

Treatments for Waldenstrom macroglobulinemia may include a "watch and wait" approach, plasma exchange, chemotherapy and targeted therapy. Other treatments may include immunotherapy and bone marrow transplant, also called bone marrow stem cell transplant. Your treatment plan depends on whether you have symptoms and the results of your blood tests and biopsies. Your healthcare team also considers how quickly the cancer is growing, your overall health and what you prefer.

Watch and wait

If immunoglobulin M (IgM) proteins are in the blood, but there are no symptoms, you may not need treatment right away. Instead, you might have blood tests every few months to monitor your condition. Healthcare professionals sometimes call this watchful waiting. Treatment may not be needed for years.

Plasma exchange

Plasma exchange, also known as plasmapheresis, removes IgM proteins from the blood. It replaces them with healthy blood plasma. Plasma exchange may be used if your healthcare professional suspects hyperviscosity syndrome. It can ease symptoms caused by having too many IgM proteins in the blood.

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.

Targeted therapy may be used to treat Waldenstrom macroglobulinemia. Targeted therapy may be combined with immunotherapy. Targeted therapy may be used when other treatments haven't worked, called refractory Waldenstrom macroglobulinemia. It also may be used for Waldenstrom macroglobulinemia that comes back after treatment, called relapsed Waldenstrom macroglobulinemia.

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 to treat Waldenstrom macroglobulinemia. It is usually combined with immunotherapy. Chemotherapy also may be used for relapsed or refractory Waldenstrom macroglobulinemia.

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 to treat Waldenstrom macroglobulinemia. It may be combined with chemotherapy or targeted therapy. Immunotherapy also may be used for relapsed or refractory Waldenstrom macroglobulinemia.

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. Stem cells can come from your own body, called an autologous transplant. Stem cells also can come from a donor, called an allogeneic transplant.

A bone marrow transplant may be an option for some relapsed or refractory Waldenstrom macroglobulinemia. Chemotherapy is typically done before the transplant to suppress the immune system and bone marrow.

Monitoring after treatment

After treatment is complete, you may have frequent follow-up appointments to see if the cancer has come back, known as a relapse. You may have repeat blood tests to check for a relapse.

With time, you'll likely find what helps you cope with the uncertainty and worry of a cancer diagnosis. Until then, these ideas may help you decide what to do next.

Learn enough about Waldenstrom macroglobulinemia 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 Waldenstrom macroglobulinemia, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with your Waldenstrom macroglobulinemia. 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 offer 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 person 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 International Waldenstrom's Macroglobulinemia Foundation, the National Cancer Institute, the American Cancer Society, Blood Cancer United and the Lymphoma Research Foundation. Find support online through Mayo Clinic Connect, which is a community where you can connect with others for support, practical information and answers to everyday questions.

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

If your healthcare professional thinks you might have Waldenstrom macroglobulinemia, you may be referred to a doctor who specializes in diseases of the blood and bone marrow, called a hematologist.

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, 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.

For Waldenstrom macroglobulinemia, some basic questions include:

• Do I have Waldenstrom macroglobulinemia?
• What is the stage of my Waldenstrom macroglobulinemia?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• What treatment options have shown the best results?
• 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?
• What will determine whether I should plan for a follow-up visit?

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 make symptoms worse?
• Have you had fevers, night sweats or weight loss?
• Have you noticed any lumps, swelling or pain anywhere in your body?
• Do you have other medical conditions?
• Has anyone in your family had lymphoma?
• What are your biggest worries about your diagnosis or treatment?

Waldenstrom macroglobulinemia survival rates are given by risk group. Other cancers might give survival rates by stage, but stages don't apply to Waldenstrom macroglobulinemia. Instead, healthcare professionals use risk groups to describe the seriousness of the cancer.

To decide on the risk group, the healthcare team uses the person's age and two blood tests, albumin and lactate dehydrogenase (LDH). People in the lower risk groups tend to live longer, while those in higher risk groups have a higher chance of dying of Waldenstrom macroglobulinemia.

Survival rates show the percentage of people with this cancer who are still alive a certain number of years after diagnosis. For example, a five-year survival rate means the percentage of people living five years after diagnosis.

The estimated five-year survival rates for each risk group:

• Low risk: about 93%.
• Low-intermediate risk: about 82%.
• Intermediate risk: about 69%.
• High risk: about 55%.

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