You may have a treatment called plasma exchange. It removes your abnormal plasma and replaces it with normal plasma from a healthy donor. Plasma is the liquid part of blood that contains blood cells and platelets. Plasma exchange also replaces the missing enzyme.
The procedure is done as follows:
First, you have your blood drawn as if donating blood.
As the blood is passed through a machine that separates blood into its different parts, the abnormal plasma is removed and your blood cells are saved.
Your blood cells are then combined with normal plasma from a donor, and then given back to you.
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 medicines that suppress the immune system, such as steroids or rituximab
Most people who undergo plasma exchange recover completely. But some people die of this disease, especially if it is not diagnosed right away. In people who do not recover, this condition can become long-term (chronic).
Call your health care provider if you have any unexplained bleeding.
Because the cause is unknown, there is no known way to prevent this condition.
McCrae KR, Sadler JE, Cines DB. Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 136.
Rita Nanda, MD, Assistant Professor of Medicine, Section of Hematology/Oncology, University of Chicago Medicine, Chicago, IL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.