High titers of inhibitors of von Willebrand factor-cleaving metalloproteinase in a fatal case of acute thrombotic thrombocytopenic purpura
โ Scribed by Han-Mou Tsai
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 140 KB
- Volume
- 65
- Category
- Article
- ISSN
- 0361-8609
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## Abstract Decreased von Willebrand factor (VWF)โcleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpuraโhemolytic uremic syndrome (UpshawโSchulman syndrome) and associated with mutations within the __ADAMTS13__ gene. In this report, we
Extensive microvascular platelet aggregation is characteristic of thrombotic thrombocytopenic purpura (TTP). Previous studies have indicated that abnormalities of von Willebrand factor (vWf) are often present in TTP patient plasma. There has not been previously any direct evidence linking these abno