Clinical usefulness of a functional assay for the von Willebrand factor cleaving protease (ADAMTS 13) and its inhibitor in a patient with thrombotic thrombocytopenic purpura

Dissociation between the level of von Wi

Dissociation between the level of von Willebrand factor-cleaving protease activity and disease in a patient with congenital thrombotic thrombocytopenic purpura

✍ Carolyn E. Snider; Jane C. Moore; Theodore E. Warkentin; Clara N. Finch; Catheri 📂 Article 📅 2004 🏛 John Wiley and Sons 🌐 English ⚖ 104 KB 👁 1 views

## Abstract Decreased von Willebrand factor (VWF)‐cleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (Upshaw‐Schulman syndrome) and associated with mutations within the __ADAMTS13__ gene. In this report, we