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Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma

✍ Scribed by K Kentouche; U Budde; M Furlan; V Scharfe; R Schneppenheim; F Zintl


Book ID
114813042
Publisher
John Wiley and Sons
Year
2007
Tongue
English
Weight
153 KB
Volume
91
Category
Article
ISSN
0803-5253

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## 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