Causes

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

• Bleeding of the gums
• Blurred or decreased vision
• Dizziness
• Easy bruising of the skin
• Fatigue
• Headache
• Mental status changes
• Nosebleeds
• Numbness, tingling, or burning pain in the hands, feet, fingers, toes, ears, or nose
• Rash
• Unintentional weight loss
• Vision loss in one eye

Additional symptoms that may be associated with this disease:

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

Exams 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 blood cells 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 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.

Outlook (Prognosis)

The average survival is about 6.5 years. Some people live more than 10 years.

In some people, the disorder may produce few symptoms and progress slowly.

Possible Complications

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

When to Contact a Medical Professional

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

Alternative Names

Waldenstrom's macroglobulinemia; Macroglobulinemia - primary; Lymphoplasmacytic lymphoma

References

Treon SP, Hatjiharissi E, Leleu X, Roccaro A, Merlini G. Waldenstrom Macroglobulinemia/Lymphoplasmacytic Lymphoma. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 88.

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

Update Date: 4/30/2012

Reviewed by: Todd Gersten, M.D., Hematology/Oncology, Palm Beach Cancer Institute, West Palm Beach, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.