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Around 80% of TTP is induced by deficient exercise of ADAMTS13. ADAMTS13 deficiency is usually constitutive, on account of biallelic ADAMTS13 pathogenic variants; or acquired, because of an inhibitory antibody. Evaluation of ADAMTS13 action is performed using exams based on the aptitude of the protease to cleave standard VWF multimers in vitro (e.A