Macroglobulinemia - primary

Alternative names
Waldenstrom’s macroglobulinemia; Macroglobulinemia of Waldenstrom

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

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  
  • 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:       o Numbness or tingling       o 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 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 in urine  
  • 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 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.

Johns Hopkins patient information

Last revised: December 6, 2012
by Simon D. Mitin, M.D.

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