Macroglobulinemia of Waldenstrom

Macroglobulinemia of Waldenstrom

Definition:

Macroglobulinemia of Waldenstrom is a cancer of the B lymphocytes (a type of white blood cell) which causes overproduction of monoclonal macroglobulin (IgM antibody).

Alternative Names:

Waldenstrom's macroglobulinemia; Macroglobulinemia - primary

Causes, incidence, and risk factors:

The cause of this disease is unknown. It is characterized by overproduction of IgM, which causes the blood to become too thick (hyperviscosity).

This hyperviscosity interferes with blood flow through small blood vessels, which leads to many of the symptoms of the disease. The disease occurs in 5 out of 100,000 people over 50 years old.

Symptoms:

fatigue
easy bruising of the skin
rash
nosebleeds (epistaxis)
bleeding of the gums
weight loss
vision loss in one eye
headache
dizziness
blurred vision
decreased vision
mental status changes
weight loss
peripheral neuropathy (changes in the nerves affecting the hands, feet, fingers, toes, ears, or nose) manifested by:
- numbness
- tingling
- burning pain

Additional symptoms that may be associated with this disease:

Signs and tests:

A physical examination may reveal enlarged spleen, liver, and lymph nodes. A retinal eye examination (examination of the eye with an ophthalmoscope) may reveal enlarged (engorged) retinal veins or retinal bleeding (hemorrhages).

A CBC shows low red blood cell count and platelet count.
Serum protein electrophoresis shows a monoclonal spike (increased amount of a monoclonal protein). This may be seen as a serum immunoelectrophoresis that shows an IgM spike or a serum immunofixation that shows an IgM spike.
Urine immunoelectrophoresis shows IgM or a urine test for urine Bence-Jones proteins (quantitative) may be positive.

Bone lesions are very rare. If they are present, a bone marrow examination will show cells that resemble both lymphocytes and plasma cells. A blood chemistry shows evidence of renal disease (kidney disease).

This disease may also alter the results of the following tests:

24-hour urine protein
total protein
serum globulin electrophoresis
positive immunofixation - urine (possibly)
T (thymus derived) lymphocyte count

Treatment:

Apheresis (in this case, it is called plasmapheresis or plasma exchange) is a procedure for removing unwanted substances from the blood. In macroglobulinemia, it is used to control the symptoms caused by hyperviscosity by removing or reducing the high concentration of IgM.

Blood is withdrawn from the person in the same way as for a blood donation, and the blood is passed through a cell separator that removes the plasma (the fluid portion, which contains the antibodies).

The formed elements (blood cells) are reconstituted with a plasma substitute or with donated plasma. The reconstituted blood is then returned to the body as in a blood transfusion. This is an acute procedure designed to quickly control symptoms.

Drug therapy may include Leukeran, Alkeran, Cytoxan, or corticosteroids.

Anemia (deficient number of red blood cells), leukopenia (deficient number of white blood cells) and thrombocytopenia (deficient number of platelets) in the later stages of the disease may require the use of packed red cell transfusions, antibiotics, or platelet transfusions.

Expectations (prognosis):

The median (average) survival is about 5 years. In some people, the disorder may be chronic with few symptoms and slow progression.

Complications:

vision impairment
gastrointestinal bleeding
alterations in mental status possibly progressing to coma
congestive heart failure

Calling your health care provider:

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

Review Date: 8/23/2002
Reviewed By: Scott Howard, M.D., M.S., Memphis, TN. Review provided by VeriMed Healthcare Network.

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