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Alternative names Return to top
TTPDefinition Return to top
TTP is a blood disorder characterized by low platelets, low red blood cell count (caused by premature breakdown of the cells), abnormalities in kidney function, and neurological abnormalities. A similar clinical condition with overlapping features is hemolytic-uremic syndrome.Causes, incidence, and risk factors Return to top
This disease is caused by a deficiency in the von Willebrand Factor cleaving protease, known as ADAMTS13. The loss of this enzyme results in large complexes of von Willbrand factor circulating in the blood, which in turn causes platelet clumping and red blood cell destruction.
Although most cases have no known cause, some cases may be related to a deficiency of ADAMTS13 at birth, cancer, chemotherapy, HIV infection, hormone replacement therapy and estrogens, and a number of medications in common use (including ticlopidine, clopidogrel, and cyclosporine A).
Symptoms Return to top
Signs and tests Return to top
Treatment Return to top
Plasmapheresis, or plasma exchange, is used to remove unwanted substances from the blood (large von Willebran protein complexes), and to replace the missing protease. Blood is withdrawn from the patient as for a blood donation.
Then the plasma portion of the blood is passed through a cell separator. The remaining portion of the blood is saved, reconstituted with fresh plasma, and returned to the patient as a blood transfusion.
In thrombotic thrombocytopenic purpura, this treatment is repeated daily until blood tests show improvement. People who do not respond to this treatment, or have frequent recurrences, may require the removal of the spleen, corticosteroids, or other immune suppression.
Expectations (prognosis) Return to top
Plasmapheresis has improved the outcome of this disease so that 80 to 90% of patients now recover completely. However, fatalities still occur. With relapses, the remaining cases become chronic (long-term).Complications Return to top
Calling your health care provider Return to top
Call your health care provider if unusual or unexplained bleeding occurs.Prevention Return to top
Because the specific cause is unknown, specific prevention is also unknown. Update Date: 10/13/2003 Updated by: Corey Cutler, M.D., M.P.H., F.R.C.P.C, Department of Medical Oncology, Dana-Farber Cancer Institute; Instructor in Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |