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About 80% of TTP is triggered by deficient exercise of ADAMTS13. ADAMTS13 deficiency could be constitutive, due to biallelic ADAMTS13 pathogenic variants; or acquired, because of an inhibitory antibody. Analysis of ADAMTS13 exercise is performed making use of tests based upon the aptitude in the protease to cleave normal VWF multimers in vitro (e.M

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