Abstract
TTP is a rare condition characterized by micro-angiopathic thrombosis, hemolysis and ischemic organ dysfunction. In recent years, it has become clear that a deficiency of the von Willebrand factor (vWF) protease, a desintegrin and metalloproteinase with thrombospondin type 1 motif (ADAMTS-13), plays a crucial role in the pathogenesis of TTP. This deficiency results in ultra large vWF multimers that cause platelet aggregation and microthrombi. Presumably, auto-immune processes are causing the acquired deficiency of ADAMTS-13: in 87–97% of the cases autoantibodies against ADAMTS-13 are found. The mainstay of treatment of TTP consists of plasmapheresis and the addition of exogenous plasma. With this, an attempt is made to remove the auto-antibodies and to supplement the deficiencies of ADAMTS-13. Addition of prednisone is advised to inhibit any further development of auto-antibodies. With this modality, a complete remission can be reached in 85% of the patients. However, 40– 50% will experience a relapse TTP and half of these occur
Published Version
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