Alternate Names: Waldenstrom's macroglobulinemia; Macroglobulinemia - primary

Causes and Risks: The cause of this disease is unknown. It affects people over 50 years old. Overproduction of IgM causes a marked increase in the viscosity (thickness) of the blood. This hyperviscosity interferes with blood flow through small blood vessels and causes many of the symptoms of the disease. Risk factors are unknown.

The incidence is 5 out of 100,000 people.

Prevention: The cause is unknown, therefore prevention is unknown.

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

• glands, swollen
• flank pain
• skin discoloration, bluish
• fingers that change color upon pressure

Signs and Tests: A physical examination may reveal an 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 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.

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 Blood is withdrawn from the person in the same way as for a blood donation, and the blood is passed through a cell separator that removes the plasma (the fluid portion, which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

Prognosis: The median 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

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