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Roughly 80% of TTP is activated by deficient activity of ADAMTS13. ADAMTS13 deficiency might be constitutive, due to biallelic ADAMTS13 pathogenic variants; or acquired, on account of an inhibitory antibody. Evaluation of ADAMTS13 exercise is carried out applying tests based on the aptitude from the protease to cleave regular VWF multimers in vitro… Read More

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