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Macroglobulinemia of Waldenstrom

Definition

Macroglobulinemia of Waldenstrom is a cancer of the B lymphocytes (a type of white blood cell). It is associated with the overproduction of proteins called IgM antibodies.

Alternative Names

Waldenstrom's macroglobulinemia; Macroglobulinemia - primary; Lymphoplasmacytic lymphoma

Causes, incidence, and risk factors

Waldenstrom's macroglobulinemia is a result of a condition called lymphoplasmacytic lymphoma. The cause of the overproduction of the IgM antibody is unknown, but researchers believe it is made by lymphoma cells.

Overproduction of IgM causes the blood to become too thick. This is called hyperviscosity. It occasionally makes it harder for blood to flow through small blood vessels.

About 1,500 people in the United States are diagnosed with Waldenstrom's macroglobulinemia every year. Most people with this condition are over age 65, however, it may occur in younger people.

Symptoms

Additional symptoms that may be associated with this disease:

Signs and tests

A physical examination may reveal a swollen spleen, liver, and lymph nodes. An eye exam may show enlarged veins in the retina or retinal bleeding (hemorrhages).

A CBC shows a low number of red bloods and platelets. A blood chemistry shows evidence of kidney disease. A serum viscosity test can tell if the blood has become thick. Symptoms usually occur when the blood is four times thicker than normal.

A test called serum protein electrophoresis shows an increased amount of the IgM antibody. Levels seen in Waldenstrom's macroglobulinemia are generally greater than 3 g/dL.

Bone lesions are very rare. If they are present, a bone marrow examination will show cells that resemble both lymphocytes and plasma cells.

Additional tests that may be done:

Treatment

Plasmapheresis (plasma exchange) removes unwanted substances from the blood. In macroglobulinemia, it removes or reduces the high level of IgM, and is used to quickly control the symptoms caused by blood thickening.

Drug therapy may include steroids, Leukeran, Alkeran, Cytoxan, fludarabine, or rituximab, or combinations of chemotherapy drugs.

Patients who have a low number of red or white blood cells or platelets may need transfusions or antibiotics.

Expectations (prognosis)

The average survival is about 5 years. In some people, the disorder may produce few symptoms and progress slowly.

Complications

Calling your health care provider

Call your health care provider if symptoms of this disorder develop.

References

Ferri FF. Ferri’s Clinical Advisor: Instant Diagnosis and Treatment. 8th ed. St. Louis, Mo: Mosby; 2006:909.

Rajkumar SV, Kyle RA. Plasma Cell Disorders. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 198.

Review Date:4/8/2008
Reviewed By:Mark Levin, MD, Hematologist and Oncologist, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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