A sensitive ristocetin co-factor activity assay with recombinant glycoprotein Ibalpha for the diagnosis of patients with low von Willebrand factor levels.

作者: Zaverio M Ruggeri , Ileana Forza , Maria T Canciani , Pier Mannuccio Mannucci , Patrizia Marchese

DOI: 10.3324/%X

关键词:

摘要: BACKGROUND AND OBJECTIVES: The assay of ristocetin co-factor activity von Willebrand factor (VWF:RCo) is used in the screening patients with suspected Willebrand's disease (VWD), most frequent inherited bleeding disorder. A correct diagnosis VWD relies on platelet agglutination tests that have a low accuracy within and between assays. more accurate VWF:RCo would improve classification. DESIGN METHODS: We describe here an ELISA method which recombinant fragment alpha-subunit glycoprotein Ib-IX-V complex (rGPIbalpha) bound to anti-GPIbalpha monoclonal antibody immobilized onto microtiter plate wells captures plasma VWF presence ristocetin. results obtained this were compared blindly values calculated from test normal subjects (n=60) type 1 (n=8), 2A (n=16), 2B (n=13), 2M (n=17) or Vicenza (n=8) characterized by levels. RESULTS: two assays gave similar both (r=0.93), but showed higher sensitivity (0.1 versus 6.25 U/dL). repeatability reproducibility coefficients variation 9% 10%, respectively, for ELISA, as 14% 15% test. INTERPRETATION CONCLUSIONS: This can be useful identification classification it may provide distinction 2 abnormal function concentration.

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