“…Indeed, dysfunctional ADAMTS13, due to autoimmune or other causes, leads to thrombotic problems. TTP is the bestknown clinical example of ADAMTS13 dysfunction; it is a multifactorial disease where defective ADAMTS13 activity due to autoantibodies, mutations, or deficiency can result in ULVWF multimers, thrombocytopenia due to vWF-binding platelets, and multiple thrombotic microangiopathies (1,13). Acute thrombotic episodes can be triggered by other prothrombotic conditions, such as increased inflammation (17,18).…”