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Roughly eighty% of TTP is triggered by deficient action of ADAMTS13. ADAMTS13 deficiency may be constitutive, due to biallelic ADAMTS13 pathogenic variants; or acquired, due to an inhibitory antibody. Evaluation of ADAMTS13 action is carried out employing tests based upon the capability in the protease to cleave typical VWF multimers in vitro (e.Th