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Waldenstrom’s Macroglobulinemia

A type of blood cell cancer

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Updated June 18, 2014

Blood contains red cells, white cells, and platelets

Blood contains red cells, white cells, and platelets

Photo © A.D.A.M.
Waldenstrom’s macroglobulinemia is a blood cell cancer. White blood cells called B lymphocytes multiply out of control, invading the bone marrow, liver, and spleen. People over age 50 have the highest risk for this type of cancer. It is estimated that about 1,000 to 1,500 people are diagnosed each year with Waldenstrom’s macroglobulinemia in the United States. White males develop this cancer twice as often as black males or white or black females.

Waldenstrom’s macroglobulinemia is somewhat similar to two other types of cancer, multiple myeloma (plasma cell cancer) and non-Hodgkin's lymphoma (a group of cancers of lymphocytes).

The cancerous B lymphocytes in Waldenstrom’s macroglobulinemia produce excessive amounts of an antibody protein called immunoglobulin M (IgM). This makes the blood thick (hyperviscous), which affects the blood flow through smaller blood vessels to the organs of the body. This causes most of the symptoms of the disease, including nervous system, heart, and vision problems.

Risk factors

Some of the risk factors for Waldenstrom’s macroglobulinemia are:
  • Age – Age is the most significant risk factor. The disease is usually diagnosed in people over age 50.
  • Race – The disease is more common in whites than in African Americans.
  • Gender – Men are more likely to develop this cancer than are women.
  • Monoclonal gammopathy of undetermined significance (MGUS) – In MGUS, there is excess production of an antibody protein (immunoglobulin) by abnormal plasma cells. MGUS is by itself harmless, and there are no symptoms, but about 20% of people with MGUS will develop multiple myeloma or non-Hodgkin lymphoma within 20 years.

Symptoms

Waldenstrom’s macroglobulinemia is usually a slow-moving (indolent) disease, and many individuals with it have no symptoms for a long time. Others have symptoms right away. Some of the symptoms may include:
  • Tiredness and fatigue
  • Loss of appetite
  • Nervous system symptoms such as numbness, tingling, or weakness of a muscle; confusion, dizziness, headaches, stroke-like symptoms
  • Abnormal bleeding, such as frequent nosebleeds or bleeding gums, or bruising easily
  • Blurred vision or blind spots
  • Low grade fever, 100 – 101 F
  • Abnormal swelling, such as swollen lymph nodes or enlarged liver or spleen
  • Kidney problems
  • Heart problems leading to weakness, shortness of breath, and fluid buildup in body tissues
  • More frequent infections

Diagnosis

Sometimes Waldenstrom’s macroglobulinemia may be suspected from the abnormal results of a blood test given for some other reason. If symptoms are present, the first blood test done is usually a complete blood count (CBC), which would show low levels of red blood cells (anemia). Next, blood and/or urine samples will be taken and examined for the abnormal antibody protein (IgM). The blood test is called serum protein electrophoresis (SPEP) and the urine test is called urine protein electrophoresis (UPEP).

Since the excess IgM can make the blood thick (hyperviscous), the blood viscosity will be checked to see if it needs to be treated. The blood levels of beta-2-microglobulin, another protein produced by the cancer cells, will be also checked. A bone marrow biopsy (sample) will be taken to look for cancer cells.

Staging

Staging a cancer means determining its extent. However, there is no standard staging system for Waldenstrom’s macroglobulinemia as there is for most other kinds of cancer. So doctors look at the degree of anemia and the levels of IgM and beta-2-microglobulin in the blood. Having anemia and higher levels of these proteins in the blood can mean a poorer outlook for the individual.

Treatment

Waldenstrom’s macroglobulinemia can be treated, but there is no cure for it. If symptoms are present, chemotherapy with anticancer drugs is started. In recent years, the drugs Fludara (fludarabine) and Leustatin (cladribine) have become the first chemotherapy drugs given to people with Waldenstrom’s macroglobulinemia. Sometimes Cytoxan (cyclophosphamide) is added. Other commonly used chemotherapy drugs are Luekeran (chlorambucil) and prednisone, usually given together, or Adriamycin (doxorubicin). Sometimes an individual may start with one combination of drugs, then switch to another combination that is more effective.

Another drug used to treat Waldenstrom’s macroglobulinemia is Rituxan (rituximab). Some doctors may use this drug for hard-to-treat disease; others may combine it with chemotherapy drugs at the start of treatment. Campath (alemtuzumab) has also been an effective treatment, as has Velcade (bortezomib).

If IgM makes the blood thick (hypervisous), plasmapheresis may be used as a treatment. The thick liquid part of the blood (plasma) is removed and replaced with fresh liquid by a machine. Plasmapheresis is helpful in relieving symptoms, and may be used at the start of treatment while waiting for the chemotherapy drugs to begin working.

Sources:

"Waldenstrom's Macroglobulinemia." Cancer Reference Information. 03 Mar 2004. American Cancer Society. 3 Nov 2007.

"Waldenstrom's Macroglobulinemia." Index of Rare Diseases. National Organization for Rare Disorders. 3 Nov 2007.

"What is Waldenstrom's Macroglobulinemia (WM)?." International Waldenstrom's Macroglobulinemia Foundation. 3 Nov 2007.

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