Article
Thrombotic thrombocytopenic purpura associated with von Willebrand factor-cleaving protease (ADAMTS13) deficiency in children.
Seminars in thrombosis and hemostasis - 1 Mar 2006
Loirat Chantal, Veyradier Agnès, Girma Jean-Pierre, Ribba Anne-Sophie, Meyer Dominique
Abstract excerpt
The physiopathology of thrombotic thrombocytopenic purpura (TTP) has been clarified since 1998, when it was shown that TTP in adults was most often associated with an acquired deficiency of von Willebrand factor-cleaving protease (ADAMTS13) due to autoantibodies, whereas TTP in children was most often associated with a hereditary autosomal recessive severe deficiency of ADAMTS13. The hereditary form of TPP...
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