Approximately 80% of TTP is activated by deficient activity of ADAMTS13. ADAMTS13 deficiency may be constitutive, due to biallelic ADAMTS13 pathogenic variants; or obtained, as a result of an inhibitory antibody. Evaluation of ADAMTS13 action is done applying tests determined by the aptitude from the protease to cleave normal VWF multimers in vitro… Read More

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