Abstract
ADAMTS13, also known as von Willebrand factor (VWF)-cleaving protease, is a plasma enzyme that is crucial in regulating VWF activity, as it preferentially cleaves ultra-large VWF (ULVWF) multimers, which are hyperactive and potentially pro-thrombotic. Severe ADAMTS13 deficiency is associated with the development of a thrombotic microangiopathy known as thrombotic thrombocytopenic purpura (TTP). In recent years, evidence has accumulated that also mild to moderate deficiency of ADAMTS13 contributes to the development of thrombotic diseases, probably due to an increase in the concentration of ULVWF multimers. This review discusses the potential use of recombinant human ADAMTS13 (rhADAMTS13) in the treatment of clinical conditions associated with reduced levels of ADAMTS13 and/or elevated VWF to ADAMTS13 ratios. We focus on current treatment and possible replacement therapy with rhADAMTS13 in patients with severe ADAMTS13 deficiency as manifested in congenital and acquired TTP and examine the potential therapeutic use of rhADAMTS13 in cardiovascular and liver diseases and malaria, where ADAMTS13 levels are only moderately decreased.
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