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's 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 that can build up in the blood. Too much of the protein can reduce blood flow in the body and cause other problems.

Symptoms

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

When they happen, Waldenstrom macroglobulinemia symptoms may include:

• Fatigue.
• Fever.
• Weight loss.
• Night sweats.
• Numbness in the hands or feet.
• Swollen lymph nodes.
• A feeling of pain or fullness under the ribs on your left side, which may be caused by an enlarged spleen.
• Easy bruising.
• Bleeding nose or gums.
• Headache.
• Shortness of breath.
• Changes in vision.
• Confusion.

When to see a doctor

Make an appointment with your primary care provider if you have ongoing symptoms that worry you.

Causes

Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell a cell what to do. The changes tell the cells to multiply quickly. The cells continue living when healthy cells would die as part of their natural lifecycle.

In Waldenstrom macroglobulinemia, the changes happen in the white blood cells. The changes turn some of the white blood cells into cancer cells. It's not clear what causes the changes.

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. The protein is immunoglobulin M, which is also called IgM. IgM can build up in the blood. This may reduce blood flow in the body and cause other problems.

Risk factors

Factors that can increase the risk of Waldenstrom macroglobulinemia include:

• Being older. Waldenstrom macroglobulinemia can occur at any age, but it's most often found in adults 70 and older.
• Being male. Males are more likely to have Waldenstrom macroglobulinemia.
• Being white. White people are more likely to develop the disease, compared with people of other races.
• Having a family history of lymphoma. Having a relative who has Waldenstrom macroglobulinemia or another type of B-cell lymphoma might increase your risk.

Diagnosis

A physical exam, medical history and the following tests are used to diagnose Waldenstrom macroglobulinemia:

• Blood tests. Blood tests can show if there are too few healthy blood cells. Also, blood tests detect a protein made by the cancer cells. This protein is immunoglobulin M, which is also called IgM.

  Blood tests also can show how well organs are working. Results can show whether the IgM proteins are harming organs, such as the kidneys and the liver.

• Collecting a sample of bone marrow for testing. During a bone marrow biopsy, a needle is used to take some bone marrow from the hipbone. 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 can help show whether cancer has spread to other areas of the body. Imaging tests might include CT scans or positron emission tomography scans, which are also called PET scans.

Treatment

Treatment options for Waldenstrom macroglobulinemia may include:

• Watchful waiting. If IgM proteins are in the blood, but there are no symptoms, treatment might not be needed right away. Instead, you might have blood tests every few months to monitor your condition. Doctors sometimes call this watchful waiting. There might be no need for treatment 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 can relieve symptoms caused by having too many IgM proteins in the blood.
• Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells throughout the body. Chemotherapy used alone or with other medicines might be the first treatment for people who have symptoms of Waldenstrom macroglobulinemia. Also, high-dose chemotherapy can stop bone marrow from making cells and may be used to prepare for a bone marrow transplant.
• Targeted therapy. Targeted therapy uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Targeted therapy medicines might be used with other treatments, such as chemotherapy or immunotherapy.
• Immunotherapy. Immunotherapy is a treatment with medicine that helps your body's immune system to kill cancer cells. Your immune system fights off diseases by attacking germs and other cells that shouldn't be in your body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.
• Bone marrow transplant. In select instances, a bone marrow transplant, also known as a stem cell transplant, may be a treatment for Waldenstrom macroglobulinemia. During this procedure, high doses of chemotherapy wipe out the bone marrow. Healthy blood stem cells go into the body to rebuild healthy bone marrow.
• Supportive care. Supportive care, which is also called palliative care, focuses on relieving pain and other symptoms of serious illness. This extra layer of care can support you as you undergo other treatments, such as chemotherapy.

Preparing for an appointment

If you have symptoms that worry you, make an appointment with your primary care provider.

If your primary care provider thinks you have Waldenstrom macroglobulinemia, you might be referred to a specialist in treating blood and bone marrow conditions, also known as a hematologist.

Here's some information to help you get ready for your appointment.

What you can do

Take a family member or friend along to help you remember the information you get.

Make a list of:

• Your symptoms and when they began.
• All the medicines, vitamins or supplements you take, including doses.
• Questions to ask your health care provider.

Questions to ask might include:

• What might be causing my symptoms?
• Are there other possible causes?
• What tests do I need?

Questions to ask a specialist if you're referred to one include:

• Do I have Waldenstrom macroglobulinemia?
• Do I need to begin treatment right away?
• What are the goals of treatment for me?
• What treatment do you recommend?
• What are the possible side effects of treatment?
• What is the outlook for my condition?

Be sure to ask any other questions you have.

What to expect from your doctor

Your provider is likely to ask you questions, such as:

• How have your symptoms changed over time?
• Does anything make them worse or better?
• Do you have other medical conditions?
• Has anyone in your family had lymphoma?