Roughly 80% of TTP is triggered by deficient activity of ADAMTS13. ADAMTS13 deficiency can be constitutive, because of biallelic ADAMTS13 pathogenic variants; or acquired, because of an inhibitory antibody. Evaluation of ADAMTS13 action is done employing exams determined by the potential on the protease to cleave common VWF multimers in vitro (e.Ho
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