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Roughly 80% of TTP is induced by deficient exercise of ADAMTS13. ADAMTS13 deficiency is often constitutive, because of biallelic ADAMTS13 pathogenic variants; or acquired, due to an inhibitory antibody. Evaluation of ADAMTS13 activity is executed employing tests based on the aptitude on the protease to cleave regular VWF multimers in vitro (e.A res