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What

Waldenstrom’s macroglobulinemia (WM) is a rare form of cancer that affects plasma cells, which come from a type of white blood cell called B cells. These plasma cells multiply out of control and fill the bone marrow, lymph nodes, and spleen. This causes an overproduction of antibodies, specifically an antibody called IgM. Increased IgM in the blood causes the blood to thicken, which can cause symptoms of dizziness, headaches, visual problems, and the tendency to bleed and bruise easily.

Who

WM is a rare condition, accounting only for 2% of all blood cancers. WM is very rare in children. In adults, it occurs in 5 out of 100,000 people over 50 years old and the average age at diagnosis is 60 yrs-old. It is more prevalent in males than females, and there is an increased incidence in Caucasians versus African Americans.

Signs and Symptoms

Some or all of the following may be seen in people with Waldenstrom’s macroglobulinemia: - Fatigue, weight loss, anorexia - Tendency to bleed and bruise easily (due to the lack of platelets) - Anemia (low red blood cell levels) - Enlarged spleen, liver, and lymph nodes - Headaches and dizziness - Visual problems, such as blurry or decreased vision - Numbness, tingling, or pain in the arms and/or legs - Mental status changes and lethargy

Possible Causes

WM is caused by the overproduction of a specific type of protein in the body known as immunoglobin type M (IgM). IgM is a protein that normally serves a protective role against infection as part of the immune system. It is produced by a blood cell known as plasma cells which are formed in the spleen, lymph nodes, and bone marrow. In WM, plasma cells multiply out of control. As these plasma cells start increasing, the body’s immune system begins overproducing antibodies as it would during an infection. Since there is no infection, these antibodies (IgM type) accumulate in the blood. This causes the blood to thicken, which disrupts flow through small arteries and veins throughout the body, as well as causing problems in the vital organs such as the brain and eyes. The thickening of the blood is what causes many of the symptoms listed above.

Diagnosis

Most of the diagnosis can be made by recognition of the signs and symptoms listed above. Additional evidence can be obtained by blood tests including a complete blood count (CBC) looking for evidence of anemia (low red blood cell count) or thrombocytopenia (low platelet counts), as well as looking at the blood under a microscope. Other blood tests may include a protein electrophoresis, which detects levels of different blood proteins (which include immunoglobulins such as IgM) that may be elevated. Urine can also be tested for a similar protein electrophoresis. X-rays of the bones may be done to determine if there are any holes or “lytic lesions,” which occur infrequently but are due to the malignant plasma cells that release substances in the marrow that stimulate bone destruction and prevent bone formation. A bone marrow biopsy performed by a hematologist may be obtained to examine for infiltration of plasma cells and a high level of IgM in the bone marrow. In addition, an eye exam by an ophthalmologist may show enlarged veins or bleeding into the eye structures which can be seen in WM. A MRI or CT is not essential in the diagnosis of WM, but may be performed to assess the chest, spine, or abdomen for any abnormalities.

Treatment

Treatment of WM depends on the severity of thickness of the person’s blood. If the blood is too thick, emergent plasmapheresis can be performed. Plasmapheresis is a procedure in which blood is removed from the veins and circulated through a machine that separates the plasma (the liquid part which contains the high levels of IgM) from the other parts of the blood (cells, platelets.) The separated blood is then returned by intravenous (IV) route to the person along with a plasma substitute. In addition, anticancer drugs are usually given (chemotherapy) which affect the bone marrow production of cells. Biological therapy with interferon alpha, which is a type of medication that stimulates the immune system to fight cancer, can also be used to help relieve symptoms. When chemotherapy fails, a splenectomy (removal of spleen) can be performed, which removes a major source of the cells that produce IgM.

Prognosis

The average survival is approximately 5 years after treatment, however there is variability depending on the response to therapy. There is a more chronic form in which patients may survive up to 2 decades. Causes of death are usually related to progression of the cell proliferation, infection, or heart failure. Other causes include stroke, renal failure, lymphomas, and chronic myeloid leukemia.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

International Waldenstrom’s Macroglobulinemia Foundation
An outstanding website with information about WM as well as ongoing research. There is also information about available networking, volunteer opportunities, and support and links to many other useful resources.

American Cancer Society
A more detailed online guide with information about WM.

National Cancer Institute
A well written web-site designed to answer most common questions and relies on highly credible information.

Google Search for Waldenstrom's Macroglobulinemia (WM)

References and Sources

www.nlm.nih.gov/medlineplus/ency/article/000588.htm www.emedicine.com/med/topic2395.htm