Article
[Acquired idiopathic thrombotic thrombocytopenic purpura: arguments for an autoimmune disease].
Presse medicale (Paris, France : 1983) - 1 Dec 2006
Coppo Paul, Veyradier Agnès, Monge Matthieu
Abstract excerpt
Thrombotic thrombocytopenic purpura (TTP) is a severe form of thrombotic microangiopathy (TMA) characterized by systemic platelet clumping, hemolytic anemia, and multiorgan failure. TTP results from a defect in ADAMTS13, a plasma enzyme specifically involved in the cleavage of highly hemostatic unusually large (UL) von Willebrand factor (vWF) multimers into smaller and less adhesive vWF forms. Failure to degrade...
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