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Waldenstrom macroglobulinemia

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

Causes

WM is a result of a condition called lymphoplasmacytic lymphoma. This is a cancer of the white blood cells, in which the B or T immune cells start dividing rapidly. The exact cause of too much production of the IgM antibody is unknown. Production of excess IgM causes the blood to become too thick. This is called hyperviscosity. It can make it harder for blood to flow through small blood vessels.

WM is very rare. Most people with this condition are over 65 years of age. It may occur in younger people also.

Symptoms

Symptoms of WM may include any of the following:

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. 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 4 times thicker than normal.

A test called serum protein electrophoresis shows an increased level of the IgM antibody. Levels are often higher than 3 grams per deciliter (g/dL).

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

Additional tests that may be done include:

Treatment

The treatment aim is to decrease the symptoms and the risk of developing organ damage.

Plasmapheresis removes unwanted substances from the blood. In MW, it removes or reduces the high level of IgM. It also quickly controls the symptoms caused by blood thickening.

Medicines may include corticosteroids, a combination of chemotherapy medicines and the monoclonal antibody to B cells, rituximab.

People 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 5 years. Some people live more than 10 years.

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

Possible Complications

Complications of MW may include:

When to Contact a Medical Professional

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

Alternative Names

Waldenstrom macroglobulinemia; Macroglobulinemia - primary; Lymphoplasmacytic lymphoma; Monoclonal macroglobulinemia

References

National Cancer Institute. PDQ adult non-Hodgkin lymphoma treatment. Bethesda, MD. National Cancer Institute. Updated January 5, 2016. www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq. Accessed June 3, 2016.

Rajkumar SV. Plasma cell disorders. In: Goldman L, Schafer AI. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 187.

Treon SP, Merlini G. Waldenstrom macroglobulinemia and lymphoplasmacytic lymphoma. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 86.

Update Date 5/14/2016

Updated by: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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