thrombotic thrombocytopenia purpura

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Thrombotic thrombocytopenic purpura is an uncommon syndrome characterised by:

  • fever
  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • neurologic and renal abnormalities

The cause is unknown but it often follows infection or in women, oral contraception or pregnancy. Young adults from 20 to 50 years are predominantly affected, with a slight female preponderance.

There is evidence that affected patients may have a platelet agglutinating factor; be unable to produce prostacyclin, an antiplatelet substance, because of vacular endothelial cell defects; and have impaired fibrinolytic potential. In chronic relapsing TTP, patients may also have a structurally altered von Willebrand's Factor, the significance of which is unknown.

It is now believed that thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome represent a spectrum of disease.

Last reviewed 01/2021