A human monoclonal antibody against the distal carboxyl terminus of ADAMTS‐13 modulates its susceptibility to an inhibitor in thrombotic thrombocytopenic purpura

BackgroundImmune thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal thrombotic microangiopathy, resulting from a severe deficiency of plasma ADAMTS‐13 (A Disintegrin And Metalloproteinase with ThromboSpondin type 1 motif, member 13) activity. IgG‐type autoantibodies are primarily responsible for the inhibition of plasma ADAMTS‐13 activity. However, the mechanism underlying autoantibody‐mediated inhibition is not fully...