Unlike thrombosis caused by intravascular injury, atypical thrombotic syndromes without vascular injury [4] have been associated with activated aberrant ULVWF path in TTP due to severe ADAMTS13 deficiency leading to microthrombosis (i.e., hereditary TTP and acquired antibody-associated TTP) [23], activated aberrant TF path in fibrin clot disease of APL due to pathologic expression of TF in leukemic cells [42]. Here, ADAMTS13 is linked to thrombotic thrombocytopenic purpura.