Journal
NEW ENGLAND JOURNAL OF MEDICINE
Volume 381, Issue 17, Pages 1653-1662Publisher
MASSACHUSETTS MEDICAL SOC
DOI: 10.1056/NEJMra1813013
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Hereditary thrombotic thrombocytopenic purpura is an autosomal recessive disorder caused by the absence of a functional protease (ADAMTS13) that processes von Willebrand factor multimers into smaller fragments. The multimers bind to platelets and initiate abnormal clotting, thrombosis, and hemolysis.
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