Article
Dissociation between the level of von Willebrand factor-cleaving protease activity and disease in a patient with congenital thrombotic thrombocytopenic purpura.
American journal of hematology - 1 Dec 2004
Snider Carolyn E, Moore Jane C, Warkentin Theodore E, Finch Clara N, Hayward Catherine P M, Kelton John G
Abstract excerpt
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 describe longitudinal studies in a patient with congenital TTP who ultimately developed end-stage renal failure and required plasma...
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