Article
ADAMTS13 and anti-ADAMTS13 autoantibodies in thrombotic thrombocytopenic purpura - current perspectives and new treatment strategies.
Expert review of hematology - 1 Jan 2016
Tersteeg Claudia, Verhenne Sebastien, Roose Elien, Schelpe An-Sofie, Deckmyn Hans, De Meyer Simon F, Vanhoorelbeke Karen
Abstract excerpt
A deficiency in ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin type-1 repeats, member 13) is associated with thrombotic thrombocytopenic purpura (TTP). Congenital TTP is caused by a defect in the ADAMTS13 gene resulting in decreased or absent enzyme activity; acquired TTP results from autoantibodies that either inhibit the activity or increase the clearance of ADAMTS13. Despite major progress in...
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