Treatment:

Plasma exchange (plasmapheresis plus infusion of donor plasma) is used to remove the antibodies that are affecting clotting from the blood and also replace the missing enzyme.

• First, you will have your blood drawn as if you were donating blood.
• The plasma portion of the blood will be passed through a cell separator. The remaining portion of the blood will be saved.
• Plasma will be added to it, and the blood will be returned to you through a transfusion.

This treatment is repeated daily until blood tests show improvement.

People who do not respond to this treatment or whose condition often returns may need to:

• Have surgery to remove their spleen
• Get drugs that suppress the immune system, such as corticosteroids or rituximab

Expectations (prognosis):

Plasma exchange has greatly improved the outcome of this disease. Most patients now recover completely. However, some people die from this disease, especially if it is not found immediately. In people who don't recover, this condition can become long-term (chronic).

Complications:

• Kidney failure
• Low platelet count (thrombocytopenia)
• Low red blood cell count (caused by the premature breakdown of red blood cells)
• Nervous system problems
• Severe bleeding (hemorrhage)
• Stroke

Calling your health care provider:

Call your health care provider if you have any unexplained bleeding.