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What is Waldenström’s Macroglobulinemia?

Waldenström’s macroglobulinemia (WM) is a clinically recognized subset of lymphoplasmacytic lymphoma, or LPL. WM is a slow-growing and rare type of cancer that was named for the Swedish doctor who discovered the disease, Jan Gosta Waldenström.1-3

WM occurs when the body makes too much of an abnormal protein called macroglobulin (immunoglobulin M [IgM]). This extra protein causes thickening of the blood (hyperviscosity) which leads to symptoms such as excess bleeding and vision problems.3

Facts about WM:

  • In the United States, as many as 1,500 people a year are diagnosed with WM4
  • The condition is almost twice as common in men as it is in women4

What is the Role of B Cells in WM?

WM is a B-cell cancer.3 A B cell is a type of white blood cell (lymphocyte) that forms from a stem cell in bone marrow. B cells play an important role in fighting infection.5

WM most likely begins with mutations or changes to the DNA of a B cell, which causes these cells to multiply out of control. These changes take place during a cell’s maturation or development stage and cause the B cell to continue to reproduce more cancer cells. Cancerous B cells produce too much IgM, which interferes with normal cell functions.6

Abnormal WM cells tend to originate primarily in the bone marrow and crowd out healthy blood cells. When the body’s red blood cell level becomes too low, a person can develop anemia. When the white blood cell count gets too low, it is hard for people affected by WM to fight off infections.6

Although WM usually involves the blood and bone marrow, it can start almost anywhere and then spread to other parts of the body, such as the lymph nodes, spleen, or lungs.6

Causes and Risk Factors

Changes or mutations in the DNA of some lymphoma cells can cause them to produce high levels of the protein IgM. This extra production of IgM is associated with most of the symptoms of WM.7

The DNA changes found in WM cells are not usually passed on from a parent. These changes may be due to environmental causes but they often take place for no obvious reason.7

Research has shown that approximately 90% of the time, WM cells have a mutation in the MYD88 gene, which usually helps immune system cells signal each other so they can survive. In cells affected with WM, this DNA mutation may cause the gene to stay constantly “on,” which can help WM-affected cells survive longer than they otherwise would.7

In a human cell, the DNA has 23 pairs of chromosomes. Often, in people with WM, part of chromosome 6 is missing. In others with WM, another type of chromosomal defect (called translocation) occurs when a piece of one chromosome attaches to a different chromosome.7

In addition, some people with WM have significant changes or flaws in other bone marrow cells. Specific cells in the bone marrow release a hormone called interleukin-6 (IL-6). When cells produce too much IL-6, it may contribute to the development of WM.7

The risk of WM increases with age.8 The average age of diagnosis is the late 60s or early 70s.4

Signs and Symptoms

The most common symptom of WM is weakness.9 People with WM also often suffer from loss of appetite, fever, night sweats, weight loss, and numbness or a painful “pins and needles” sensation in their feet and legs (neuropathy). The disease also can also cause abnormal bleeding, vision problems, heart and kidney problems, and may cause an increased sensitivity to cold.9

Diagnosis and Treatment

If you have symptoms of WM, typically your doctor will first perform a physical exam and test your blood for the disease. Then your doctor will perform a biopsy, whereby a small amount of semi-liquid bone marrow and solid bone is removed and evaluated in order to confirm the diagnosis.9

Treatment depends on a variety of factors, including age, prognosis, and general health. Some patients do not need to be treated right away, and their condition can be monitored until they receive treatment. As with all medical decisions, it is important to discuss all options with your doctor.10

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Treatment options include10:

  • Chemotherapy—Medications that are given either orally, by injection, or by infusion to destroy or control cancer cells
  • Immunotherapy (including monoclonal antibodies)—Medications that help the body’s immune system fight cancer
  • Plasma exchange (plasmapheresis)—A treatment that is used when IgM has built up and caused the blood to thicken. A machine separates the liquid part of the blood (plasma) with the excess IgM from the blood cells. The plasma with the excess IgM is discarded. The blood cells are combined with salt solution and new plasma and returned to the patient’s blood
  • Stem cell transplant—A process by which stem cells (cells that create new undamaged cells) are removed and stored at the hospital while treatment is ongoing. After treatment, these stem cells are reintroduced (infused) into the bone marrow to help create new blood cells
  • Kinase inhibitors—Medications that block the activity of a protein called a kinase. A kinase can tell leukemia cells to divide and help them survive. By blocking this protein, kinase inhibitors can address changes inside of cells that may cause them to become cancerous

You Are Not Alone

Now is an important time to look to your healthcare providers and caregivers for support. There are also plenty of resources available if you would like to learn more about WM. Start by visiting the following Web sites*:

Advocacy Connector

The American Cancer Society (WM)

Cancer Care (non-Hodgkin lymphoma)

Cancer Support Community (non-Hodgkin lymphoma)

International Waldenstrom's Macroglobulinemia Foundation

Leukemia and Lymphoma Society (Waldenstrom's Fact Sheet)

Lymphoma Research Foundation (WM)

Patient Advocate Foundation

Powerful Patient

* These resources are an incomplete listing of cancer support organizations and are not controlled by, endorsed by, or affiliated with Pharmacyclics, Inc., or Janssen Biotech, Inc. The list is meant for informational purposes only and is not intended to replace your healthcare professional’s medical advice. Ask your doctor or oncology nurse educator any questions you may have about your cancer or treatment plan.

Glossary of Terms

Here are the definitions of terms you may come across as you learn more about WM.

Chromosomes: Threadlike parts inside cells that carry hereditary information. Chromosomes are made up of protein and DNA.

Controlled: When treatment by a doctor relieves symptoms and keeps WM from getting worse.

Lymph nodes: Small immune system organs that store the white blood cells until they are needed to fight off disease. Lymph nodes are scattered around your body in places such as the base of the neck, groin, and armpits.

Lymphocytes: White blood cells that help the body fight disease. The two main types are B cells and T cells. B cells make antibodies that attack bacteria and toxins. T cells attack cells that have been taken over by viruses (such as HIV) or have become cancerous.

Progressed: When WM becomes worse. Symptoms become more serious and signs of the disease increase.

Remission: When signs of WM go away, it is called “total remission.” When signs of WM are reduced by at least 50%, it is called a “partial remission."11

White blood cells: One of the main parts of blood. White blood cells fight disease. White blood cells are made in the bone marrow and wait in the lymph nodes until they are needed.

References:
  1. Waldenström’s macroglobulinemia: overview. Lymphoma Research Foundation Web site.
    http://www.lymphoma.org/site/apps/s/content.asp?c=bkLTKaOQLmK8E&b=629813.... Accessed October 17, 2014.
  2. Waldenström’s macroglobulinemia [definition]. Merriam-Webster Web site. 
    http://www.merriam-webster.com/medical/waldenstr%C3%B6m's%20macroglobuli.... Accessed October 17, 2014.
  3. What is Waldenstrom macroglobulinemia? American Cancer Society Web site.
    http://www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/.... Accessed October 15, 2014.
  4. Waldenström macroglobulinemia detailed guide: What are the key statistics about Waldenström macroglobulinemia? American Cancer Society Web site. 
    http://www.cancer.org/acs/groups/cid/documents/webcontent/003148-pdf.pdf. Accessed October 15, 2014.
  5. B cell [definition]. National Cancer Institute Web site. 
    http://www.cancer.gov/dictionary?cdrid=45611. Accessed October 15, 2014.
  6. Waldenström macroglobulinemia facts. Leukemia and Lymphoma Society Web site.
    https://www.lls.org/content/nationalcontent/resourcecenter/freeeducation.... Accessed October 15, 2014.
  7. Do we know what causes Waldenström macroglobulinemia? American Cancer Society Web site.
    http://www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/.... Accessed October 15, 2014.
  8. What are the risk factors for Waldenström macroglobulinemia? American Cancer Society Web site.
    http://www.cancer.org/acs/groups/cid/documents/webcontent/003148-pdf.pdf. Accessed October 15, 2014.
  9. How is Waldenström macroglobulinemia diagnosed? American Cancer Society Web site.
    http://www.cancer.org/acs/groups/cid/documents/webcontent/003148-pdf.pdf. Accessed October 15, 2014.
  10. How is Waldenström macroglobulinemia treated? American Cancer Society Web site.
    http://www.cancer.org/acs/groups/cid/documents/webcontent/003148-pdf.pdf. Accessed October 15, 2014.
  11. Buske C, Leblond V. How to manage Waldenstrom’s macroglobulinemia. Leukemia. 2013; 27:762-772.
    http://www.nature.com/leu/journal/v27/n4/pdf/leu201336a.pdf. Accessed October 23, 2014.