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Roughly eighty% of TTP is triggered by deficient exercise of ADAMTS13. ADAMTS13 deficiency is often constitutive, due to biallelic ADAMTS13 pathogenic variants; or obtained, as a result of an inhibitory antibody. Evaluation of ADAMTS13 action is carried out using tests based on the aptitude from the protease to cleave standard VWF multimers in vitr… Read More

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