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5 answers about Waldenstrom macroglobulinemia

Learn the basics about this rare form of non-Hodgkin lymphoma, including symptoms and treatment.

Doctor checking glands for potential signs of waldenstrom macroglobulinemia (WM).

Waldenstrom macroglobulinemia (WM), also known as lymphoplasmacytic lymphoma, is a type of non-Hodgkin lymphoma (NHL). It is rare, and accounts for less than 2 percent of cases of non-Hodgkin lymphoma. If you or a loved one has been diagnosed with WM, the following questions and answers may help you better understand this type of cancer, how it affects the body and what treatments are available. Remember that everyone’s experience with cancer is different, and your best source of information about your diagnosis is your healthcare provider.

What is non-Hodgkin lymphoma?

Lymphomas are cancers of the lymphatic system, a network of vessels, nodes and organs that performs several important functions for the body. One function is to filter out immune cells and excess fluids. Another is to transport lymph throughout the body. Lymph is a fluid that contains white blood cells. Lymphomas fall into two broad categories, Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). While both cancers are lymphomas, they are very different in terms of how the cancer cells are structured and how they behave in the body. There are more than 60 subtypes of NHL, one of which is Waldenstrom macroglobulinemia.

What causes WM?

As with many other forms of cancer, WM is caused by mutations in DNA that change how cells work and behave, but healthcare researchers do not know what causes these changes to occur. WM occurs when there are mutations to a type of white blood cell called B cells. These mutations cause B cells to overproduce an antibody called immunoglobulin M (IgM).

WM is most common in Caucasian men, and being over the age of 65 is considered a risk factor (the average age of diagnosis is 70). About 20 percent of people with WM have a close family member with WM or another disease that involves abnormal B cells (certain types of leukemia or lymphomas, for example). Some research suggests that hepatitis C and some autoimmune conditions may increase a person’s risk of developing WM. Having WM increases a person’s risk for other types of cancers.

What are the symptoms?

WM tends to develop slowly, and it may not cause symptoms for a number of years. Signs and symptoms of WM can vary from person to person. People who have WM may experience swollen lymph nodes, fatigue, unexplained weight loss, infections, fever and night sweats—which are symptoms seen with all types of lymphoma and other types of cancer. People with WM often have anemia, a blood disorder where a person does not have enough healthy red blood cells, which can contribute to fatigue. Other signs and symptoms of WM include blurred vision, loss of feeling and strength in the limbs, pain and/or coldness in the hands and feet, bleeding in the nose and mouth, abdominal swelling, loss of appetite, heart problems and kidney problems.

How is WM treated?

While there is no cure for WM, there are treatments that can control symptoms, prevent complications and improve the quality of life for people who have WM. Treatment goals and approaches to treatment will vary from person to person, and take into account numerous factors, including the age and overall health of the person with WM, symptoms and blood work. In cases where WM is not causing symptoms, a healthcare provider may advise a “watch and wait” approach where the cancer is closely monitored. In other instances, a person with WM may be treated with plasma exchange (plasmapheresis), chemotherapy, biologic therapies or targeted therapies. There is one drug that is specifically approved for the treatment of WM, and more that are being investigated as potential treatments for WM.

Medically reviewed in March 2019.

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