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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 |
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| Authors: | G Castaman A Tosetto A Goodeve J Batlle J Goudemand R Schneppenheim J Ingerslev F Hill F Rodeghiero |
| Institution: | a Department of Hematology, San Bortolo Hospital, Vicenza, Italy b The Academic Unit of Haematology, University of Sheffield, Sheffield, United Kingdom c Hemophilia and Thrombosis Centre, Foundation IRCCS Maggiore Policlinico Hospital, Mangiagalli Regina Elena and University of Milan, Milan, Italy d Servicio de Hematologia y Hemoterapia, Hospital Teresa Herrera, La Coruna, Spain e Institut National de la Santè et de la Recherche Médicale, INSERM U143, Paris, France f University of Lille, France g Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands h University Medical Center Hamburg-Eppendorf, Department of Pediatric Hematology and Oncology, Hamburg, Germany i Aesculabor Laboratory, Hamburg, Germany j Centre for Hemophilia and Thrombosis, University Hospital Skejby, Aarhus, Denmark k Department for Coagulation Disorders, University of Lund, Malmö, Sweden l Department of Hematology, Children's Hospital Birmingham, United Kingdom |
| Abstract: | BackgroundAccurate measurement of von Willebrand factor (VWF) is a critical requirement for the diagnosis of von Willebrand disease (VWD).Aim of the studyTo evaluate the diagnostic efficiency of a rapid quantitative test for the measurement of VWF antigen (VWF:Ag) in type 1 VWD.Patients and methodsVWF: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.ResultsThe assay linearity range was 10-125 IU/dL for LIA (R2 = 0.99) and 5-133 IU/dL for ELISA (R2 = 0.99). The inter-assay CV for low VWF levels (∼ 30 IU/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).ConclusionVWF: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 |
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