Definition

Macroglobulinemia of Waldenstrom is a cancer of the B lymphocytes (a type of white blood cell). Such cancer is called lymphoplasmacytic lymphoma. 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

The cause of the overproduction of the IgM antibody is unknown. Overproduction of IgM causes the blood to become too thick. This is called hyperviscosity. It 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

• Fatigue
• Easy bruising of the skin
• Rash
• Nosebleeds (epistaxis)
• Bleeding of the gums
• Unintentional weight loss
• Vision loss in one eye
• Headache
• Dizziness
• Blurred or decreased vision
• Mental status changes
• Peripheral neuropathy (changes in the nerves affecting the hands, feet, fingers, toes, ears, or nose) manifested by:
  • Numbness or tingling
  • Burning pain

Additional symptoms that may be associated with this disease:

• Swollen glands
• Flank pain
• Bluish skin discoloration
• Fingers that change color upon pressure

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:

• 24-hour urine protein
• Total protein
• Serum globulin electrophoresis
• Immunofixation in urine
• T (thymus derived) lymphocyte count

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.

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

Support Groups

Expectations (prognosis)

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

Complications

• Vision problems
• Gastrointestinal bleeding
• Changes in mental function, possibly leading to coma
• Congestive heart failure

Calling your health care provider

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

Prevention

Images

References

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

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 200:1068, 1992.

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