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.