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
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).
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, Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier;2008:chap 139.
Schafer A. Hemorrhagic disorders: Abnormalities of platelet and vascular function. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa:Saunders Elsevier;2007:chap 179.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.