ADAMTS13 is a metalloproteinase that cleaves von Willebrand factor (VWF) multimers in plasma into smaller forms, thereby preventing excessive platelet thrombus formation 1. The enzyme functions as a disintegrin metalloprotease with thrombospondin type 1 motifs and processes VWF multimers to inhibit their interaction with platelets and subsequent microvascular thrombosis 1. ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura (TTP), a life-threatening microangiopathy characterized by hemolytic anemia, thrombocytopenia, and end-organ damage 1. ADAMTS13 activity below 10% is indicative of TTP 1. Germline ADAMTS13 mutations account for approximately one-fifth of TTP cases, representing the hereditary form 2. Over 50 ADAMTS13 mutations causing familial TTP have been identified 3. Clinically, ADAMTS13 measurement is critical for TTP diagnosis and differential diagnosis of thrombotic microangiopathies 1. Recombinant ADAMTS13 (rADAMTS13) was recently FDA-approved for hereditary TTP treatment and reduces plasma exchange requirements and time to clinical response 4. Preclinical studies demonstrate rADAMTS13 efficacy in treating arterial thrombosis and inflammation without causing bleeding, suggesting broader antithrombotic applications 5. Current therapeutic development focuses on enhancing ADAMTS13 stability against proteolytic degradation 6.