Performance and utility of a cost-effective collagen-binding assay for the laboratory diagnosis of Von Willebrand disease

2007 ◽  
Vol 45 (8) ◽  
Author(s):  
Muriel Meiring ◽  
Philip N. Badenhorst ◽  
Mareli Kelderman
Keyword(s):  
Binding Assay ◽  
Cost Effective ◽  
Laboratory Diagnosis ◽  
Von Willebrand Disease ◽  
Collagen Binding ◽  
Von Willebrand

scholarly journals Performance and Clinical Utility of a Commercial von Willebrand Factor Collagen Binding Assay for Laboratory Diagnosis of von Willebrand Disease

2006 ◽  
Vol 52 (10) ◽  
pp. 1965-1967 ◽  
Author(s):  
Joanna Popov ◽  
Olga Zhukov ◽  
Susan Ruden ◽  
Terry Zeschmann ◽  
Anthony Sferruzza ◽  
...  
Keyword(s):  
Binding Assay ◽  
Binding Capacity ◽  
Laboratory Diagnosis ◽  
Von Willebrand Disease ◽  
Collagen Binding ◽  
Von Willebrand ◽  
Ristocetin Cofactor ◽  
Probable Type

Abstract Background: Von Willebrand disease (VWD) diagnosis and classification usually require a combination of nonspecific and VW-factor (VWF)-specific assays. We evaluated the analytical performance of a commercially available collagen-binding assay (CBA) and its usefulness in conjunction with other assays for laboratory diagnosis of VWD. Methods: We used a commercial CBA ELISA (Life Technologies) to evaluate 3085 plasma samples. We used standard procedures to perform other assays, including factor VIII activity (FVIII:C), VWF antigen (VWF:Ag), ristocetin cofactor activity, VWF collagen binding capacity (VWF:CB), and VWF multimeric analysis. Results: CBA intra- and interassay CVs were <6% and <13%, respectively. Reference intervals were 45%–198% for VWF:CB and 0.75–1.32 for the VWF:CB/Ag ratio. Of 3085 samples tested, 235 (8%) had results commonly associated with VWD. Multimer analysis and phenotypic data in 156 samples identified VWD types as: 91 (58%) type 1, 62 (40%) type 2, and 3 (2%) type 3. Of the 91 type 1 samples, proportional decreases in functional activity were seen in 75 samples (82%) according to CBA and in 63 samples (69%) according to the ristocetin cofactor assay. Of the type 2 samples, 10 were further identified as probable type 2A, 26 as probable type 2B, 12 as probable type 2M, and 14 could not be subtyped. VWF:CBA/Ag ratios <0.5 occurred in 83% of VWD type 2A and 2B samples, indicating characteristic functional discordance. Mean (SD) VWF:CB values were significantly higher in individuals without group O blood [113 (45)] than in those with group O blood [83 (32)] (t-test, P = 0.007). Conclusions: The commercial CBA assay produces reliable results and is useful for laboratory diagnosis of VWD.

Identification and Functional Characterisation of von Willebrand Disease

2009 ◽  
Vol 03 (01) ◽  
pp. 25
Author(s):  
Emmanuel J Favaloro ◽  
Keyword(s):  
Von Willebrand Factor ◽  
Classification Scheme ◽  
Binding Assay ◽  
Von Willebrand Disease ◽  
Collagen Binding ◽  
Clinical Uncertainty ◽  
Functional Characterisation ◽  
Diagnostic Approaches ◽  
Von Willebrand ◽  
Willebrand Factor

The identification and functional characterisation of von Willebrand disease (VWD) is challenging due to clinical uncertainty and limitations in test processes and panels used by laboratories, and because the classification scheme does not always permit unequivocal assignment of subtype. This article reviews contemporary alternatives to classic diagnostic approaches, including the incorporation of extended core test panels inclusive of the collagen-binding assay and the potential for desmopressin (DDAVP) challenge not only to provide therapeutic information but also to assist the better characterisation of individuals with defects or deficiencies in von Willebrand factor (VWF). Supplementary assays such as the PFA-100® and the VWF propeptide assay following DDAVP challenge are also worth considering.

von Willebrand Disease in a Pediatric-based Population - Comparison of Type 1 Diagnostic Criteria and Use of the PFA-100® and a von Willebrand Factor/Collagen-binding Assay

2000 ◽  
Vol 84 (09) ◽  
pp. 401-409 ◽  
Author(s):  
J.A. Dean ◽  
V. S. Blanchette ◽  
M. D. Carcao ◽  
A. M. Stain ◽  
C. R. Sparling ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Von Willebrand Factor ◽  
Screening Test ◽  
Binding Assay ◽  
Von Willebrand Disease ◽  
Consensus Guidelines ◽  
Collagen Binding ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryDefinitive diagnosis of type 1 von Willebrand Disease (VWD) remains a problem. Provisional consensus guidelines for the diagnosis of definite and possible type 1 VWD were prepared by the Scientific Subcommittee on von Willebrand factor (VWF) of the Scientific and Standardization Committee (SSC) of the International Society on Thrombosis and Haemostasis (ISTH) during the 1996 annual meeting for the specific purpose of further evaluation in retrospective and prospective studies by a Working Party on Diagnostic Criteria (1996 Annual Report of the SSC/ISTH Subcommittee on VWF). In the first phase of this study, we compared 2 definitions of type 1 VWD, each with 3 criteria: significant bleeding history, laboratory investigations, and family history. Using the ISTH consensus guidelines for type 1 VWD definition, significantly fewer patients were diagnosed with definite type 1 disease as compared to our “in house” Hospital for Sick Children (HSC) criteria (4 vs. 31). While we recognize that the provisional ISTH consensus guidelines were not intended for clinical use, we believe that the results of our studies are of interest and will assist in any future refinements to the ISTH guidelines.In the second phase of this study, we investigated the utility of 2 new tests, a laboratory screening test and a functional test, for VWD in our well characterized, pediatric-based population. The Platelet Function Analyzer (PFA-100®) provides an in vitro measure of primary hemostasis under conditions of high shear, using disposable cartridges containing collagen and either epinephrine or ADP. All tested subjects with types 2 or 3 VWD had prolonged PFA-100 closure times (CTs) with both cartridge types (n = 17) and prolonged bleeding times (n = 14). In subjects with definite type 1 VWD, 20/24 (83%) had prolonged CTs with the collagen/ADP cartridge (19/24 (79%) with collagen/epinephrine), compared with 7/26 (27%) with prolonged bleeding times. In subjects with definite types 1, 2, or 3 VWD, collagen/ADP CTs were abnormal in 37/41 subjects, giving an overall sensitivity of 90%. With this high sensitivity, the PFA-100 is a better screening test for VWD than the bleeding time.We also tested a VWF collagen-binding assay (VWF:CBA) as a functional test for VWF, in comparison with the more routinely-used ristocetin cofactor assay (VWF:RCo). The VWF:CBA is based on an ELISA technique, which has the potential to be more reproducible than the VWF:RCo. We found that the VWF:CBA detected 43/49 (88%) subjects with definite types 1, 2, or 3 VWD, performing as well as the VWF:RCo, that detected 42/48 (88%). We also showed that, used in conjunction with VWF antigen levels, the VWF:CBA may be useful in classification of VWD subtypes.

Identification and Functional Characterization of von Willebrand Disease

2009 ◽  
Vol 02 ◽  
pp. 16
Author(s):  
Emmanuel J Favaloro ◽  
Keyword(s):  
Von Willebrand Factor ◽  
Classification Scheme ◽  
Binding Assay ◽  
Functional Characterization ◽  
Von Willebrand Disease ◽  
Collagen Binding ◽  
Diagnostic Approaches ◽  
Von Willebrand ◽  
Willebrand Factor

The identification and functional characterization of von Willebrand disease (VWD) is challenging due to clinical uncertainty and limitations in test processes and panels used by laboratories, and because the classification scheme does not always permit unequivocal assignment of subtype. This article reviews contemporary alternatives to classic diagnostic approaches, including the incorporation of extended core test panels inclusive of the collagen-binding assay, and the potential for desmopressin (DDAVP) challenges not only to provide therapeutic information but also to assist the better characterization of individuals with defects or deficiencies in von Willebrand factor (VWF). Supplementary assays such as the PFA-100® and the VWF propeptide assay following DDAVP challenge are also worth considering.

von Willebrand factor collagen binding assay in von Willebrand disease type 2A, 2B, and 2M

2006 ◽  
Vol 4 (9) ◽  
pp. 2088-2090 ◽  
Author(s):  
L. BARONCIANI ◽  
A. B. FEDERICI ◽  
G. COZZI ◽  
M. T. CANCIANI ◽  
P. M. MANNUCCI
Keyword(s):  
Von Willebrand Factor ◽  
Binding Assay ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Collagen Binding ◽  
Von Willebrand ◽  
Willebrand Factor
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