Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the European multicenter study MCMDM-1VWD

Castaman, G.; Tosetto, A.; Cappelletti, A.; Goodeve, A.; Federici, A.B.; Batlle, J.; Meyer, D.; Goudemand, J.; Eikenboom, J.C.J.; Schneppenheim, R.; Budde, U.; Ingerslev, J.; Lethagen, S.; Hill, F.; Peake, I.R.; Rodeghiero, F.

doi:10.1016/j.thromres.2010.06.013

« Previous Next »Thrombosis Research
Volume 126, Issue 3 , Pages 227-231, September 2010

Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the European multicenter study MCMDM-1VWD

G. Castaman
- Department of Hematology, San Bortolo Hospital, Vicenza, Italy
- Corresponding author. Department of Cellular Therapies and Hematology, San Bortolo Hospital, I-36100 Vicenza, Italy. Tel.: +39 444 753679; fax: +39 444 753922.
A. Tosetto
- Department of Hematology, San Bortolo Hospital, Vicenza, Italy
A. Cappelletti
- Department of Hematology, San Bortolo Hospital, Vicenza, Italy
A. Goodeve
- The Academic Unit of Haematology, University of Sheffield, Sheffield, United Kingdom
A.B. Federici
- Hemophilia and Thrombosis Centre, Foundation IRCCS Maggiore Policlinico Hospital, Mangiagalli Regina Elena and University of Milan, Milan, Italy
J. Batlle
- Servicio de Hematologia y Hemoterapia, Hospital Teresa Herrera, La Coruna, Spain
D. Meyer
- Institut National de la Santè et de la Recherche Médicale, INSERM U143, Paris, France
J. Goudemand
- University of Lille, France
J.C.J. Eikenboom
- Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands
R. Schneppenheim
- University Medical Center Hamburg-Eppendorf, Department of Pediatric Hematology and Oncology, Hamburg, Germany
U. Budde
- Aesculabor Laboratory, Hamburg, Germany
J. Ingerslev
- Centre for Hemophilia and Thrombosis, University Hospital Skejby, Aarhus, Denmark
S. Lethagen
- Department for Coagulation Disorders, University of Lund, Malmö, Sweden
F. Hill
- Department of Hematology, Children's Hospital Birmingham, United Kingdom
I.R. Peake
- The Academic Unit of Haematology, University of Sheffield, Sheffield, United Kingdom
F. Rodeghiero
- Department of Hematology, San Bortolo Hospital, Vicenza, Italy

Received 17 February 2010; received in revised form 16 June 2010; accepted 17 June 2010. published online 23 July 2010.

Abstract

Background

Accurate measurement of von Willebrand factor (VWF) is a critical requirement for the diagnosis of von Willebrand disease (VWD).

Aim of the study

To evaluate the diagnostic efficiency of a rapid quantitative test for the measurement of VWF antigen (VWF:Ag) in type 1 VWD.

Patients and methods

VWF:Ag was measured with an ELISA in a robotic instrument, as a reference method, and with a fully automated latex-immunoassay (LIA) on an ACL 9000 analyser in 1,716 subjects enrolled within the Molecular and Clinical Markers for Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD) Study. Among these subjects, 1,049 were healthy controls, 281 healthy family members and 386 affected members from 127 European families with type 1 VWD.

Results

The assay linearity range was 10-125IU/dL for LIA (R²=0.99) and 5-133IU/dL for ELISA (R²=0.99). The inter-assay CV for low VWF levels (∼30IU/dL) was 2% for the LIA test and 8.7 % for ELISA. The sensitivity for detection of type 1 VWD affected members was 86% and the specificity 91% for LIA, 87% and 90% for ELISA. A receiver-operator (ROC) analysis disclosed only a marginal difference between the two tests, LIA having a slightly greater area under the curve (0.94 vs. 0.93, p=0.03).

Conclusion

VWF:Ag LIA compared well to standard ELISA in this large population of patients and controls, showing better CV. However the lower detection limit for the VWF:Ag LIA compared to the VWF:Ag ELISA means that the LIA assay is less good at discriminating between type 3 VWD and moderate type 1 VWD.

Keywords: von Willebrand factor, von Willebrand disease, Inherited bleeding disorders