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Around 80% of TTP is activated by deficient exercise of ADAMTS13. ADAMTS13 deficiency can be constitutive, as a result of biallelic ADAMTS13 pathogenic variants; or acquired, as a result of an inhibitory antibody. Evaluation of ADAMTS13 exercise is executed using tests based on the aptitude in the protease to cleave standard VWF multimers in vitro

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