Thrombotic thrombocytopenic purpura

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Thrombotic thrombocytopenic purpura

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 (nervous system) abnormalities. A similar condition is hemolytic-uremic syndrome.

In some cases, this disease is caused by a deficiency in an enzyme (a type of protein) called the von Willebrand Factor cleaving protease (also called ADAMTS13). 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).

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