The landscape of ADAMTS13-related tests in thrombotic thrombocytopenic purpura: a review

Thrombotic thrombocytopenic purpura (TTP), an insidious and often devastating thrombotic microangiopathy, represents one of the few true hematologic emergencies. Since the identification of the von Willebrand factor (VWF) cleaving protease ADAMTS13 in the early 21st century, tremendous advances have been made in the diagnosis and treatment of the disease. However, as with many areas of science, new knowledge begets new questions about the pathophysiology of both congenital and immune TTP, which identify gaps in our understanding of the mechanistic effects of ADAMTS13 and its inhibitors. Improvements in the clinical management of patients that have—or are suspected to have—TTP requires further advances in diagnostic testing and identification of clinically relevant biomarkers. In this review, we discuss ADAMTS13-related diagnostic testing in TTP, identify the underlying assumptions that guide the field, and explore avenues of research that promise to improve the lives of patients with the disease.