ADAMTS13 and thrombotic thrombocytopenic purpura: HNPs can also trigger the secretion of platelet-granule contents, especially soluble CD154, and inhibit fibrinolysis, leading to a procoagulant state.20Changes of VWF secretion, multimer distributions, and plasma levels might also trigger TTP.19, 21, 22Some proteins might counterbalance ADAMTS13 deficiency, which might explain why our patient had no recurrent iTTP episodes between 1985 and 2014.