Clinical use of a rapid collagen binding assay for von Willebrand factor cleaving protease in patients with thrombotic thrombocytopenic purpura. Thromb Haemost 2002 Oct;88(4):598-604
Date
10/04/2002Pubmed ID
12362230Scopus ID
2-s2.0-0036797613 (requires institutional sign-in at Scopus site) 47 CitationsAbstract
A simple collagen binding assay (CBA) for measuring activity of the von Willebrand factor cleaving protease in clinical samples is described, and results of fifty masked plasmapheresis samples rom patients with TTP/HUS and other diseases are presented. There was 97.5% concordance between the CBA and a multimer gel assay. The CBA identified low protease activity in 78% of patients who had a clinical syndrome consistent with TTP/HUS and in 2 of 10 sick controls, giving it a positive predictive value of 0.94. The heterogeneity regarding the presence or absence of vWF protease activity in patients with TTP/HUS was confirmed by finding a low negative predictive value of 0.50 with the CBA. The CBA detected inhibitors of the protease in 26 of 29 patients (90%) with TTP/HUS and low protease activity levels. The CBA is a useful clinical assay for examining von Willebrand factor protease activity and detecting inhibitors against the protease.
Author List
Rick ME, Moll S, Taylor MA, Krizek DM, White GC 2nd, Aronson DLAuthor
Gilbert C. White MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
ADAM ProteinsADAMTS13 Protein
Adult
Autoantibodies
Case-Control Studies
Clinical Enzyme Tests
Collagen
Dimerization
Electrophoresis, Agar Gel
Hemolytic-Uremic Syndrome
Humans
Metalloendopeptidases
Middle Aged
Plasmapheresis
Predictive Value of Tests
Protein Binding
Purpura, Thrombotic Thrombocytopenic
