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Close to 80% of TTP is induced by deficient action of ADAMTS13. ADAMTS13 deficiency can be constitutive, due to biallelic ADAMTS13 pathogenic variants; or obtained, as a result of an inhibitory antibody. Evaluation of ADAMTS13 action is done using exams based on the capability in the protease to cleave standard VWF multimers in vitro (e.Modular AHU