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ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw‐Schulman syndrome

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Hereditary thrombotic thrombocytopenic purpura, also called Upshaw‐Schulman syndrome (USS), is a rare disease caused by genetic mutations in the ADAMTS13 gene, which severely decrease the activity of ADAMTS13, a metalloprotease… Click to show full abstract

Hereditary thrombotic thrombocytopenic purpura, also called Upshaw‐Schulman syndrome (USS), is a rare disease caused by genetic mutations in the ADAMTS13 gene, which severely decrease the activity of ADAMTS13, a metalloprotease that cleaves von Willebrand factor multimers (VWF). Genotypically identical patients can show great phenotypic diversity.

Keywords: adamts13 kinetics; kinetics therapeutic; upshaw schulman; therapeutic plasma; schulman syndrome

Journal Title: Journal of Clinical Apheresis
Year Published: 2019

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