title: Laboratory diagnosis of von Willebrand's disorder: quality and diagnostic improvements driven by peer review in a multilaboratory test process.
abstract: Regular multilaboratory surveys of laboratories derived primarily from Australia, New Zealand and Southeast Asia have been conducted over the past 7 years to evaluate testing proficiency in the diagnosis of von Willebrand's disorder (VWD) and to assess changes to test practice. Participating laboratories (currently 45) are asked to perform their usual panel of tests for VWD, and then to self-interpret test results as to the likelihood (or not) of VWD, as well as to the potential subtype identified. Samples provided in the past two survey distributions (both conducted in 2003) were as follows. Survey part A/distribution 1: Normal donor plasma, plasma with borderline normal/reduced levels of VWF (x2) and plasma from an individual with type 2 A VWD. Survey part B/distribution 2 (family VWD study): Plasma from a father, mother and son with borderline normal/reduced von Willebrand factor (VWF), and a daughter with type 3 VWD. In line with previously published survey results, the interassay and within method coefficients of variation (CV) were similar for all assays (around 15-25%), although tending to be slightly higher for VWF:RCo and VWF:CB than VWF:Ag and FVIII:C. Most laboratories reported test values consistent with expected findings, and made correct interpretations or predictions regarding the nature of the samples, although discrepant assay results or interpretations are still seen in approximately 5-10% of responses (typically from laboratories using a more limited test panel or not performing the VWF:CB). Overall, problems with the non-identification of functional VWF discordance in type 2 VWD, the misidentification of functional VWF discordance in type 1 VWD, and difficulties in discriminating types 1 and 3 VWD appear to predominate. In comparison with previous surveys, performance of electro-immuno diffusion (EID) (or Laurel gel) procedures has now ceased, and a reduction in VWF:RCo and VWF:Multimer testing and an increase in latex immunoassay (LIA) testing is sustained. We conclude that laboratories are generally proficient in tests for VWD, and that diagnostic error rates are reduced when test panels are more comprehensive and include the VWF:CB.
authors.affiliation: Department of Haematology, Institute of Clinical Pathology and Medical Research, Westmead Hospital, NSW, Australia. emmanuel@icpmr.wsahs.nsw.gov.au
authors.forename: E J
authors.initials: EJ
authors.lastname: Favaloro
authors.forename: R
authors.initials: R
authors.lastname: Bonar
authors.forename: G
authors.initials: G
authors.lastname: Kershaw
authors.forename: J
authors.initials: J
authors.lastname: Sioufi
authors.forename: M
authors.initials: M
authors.lastname: Hertzberg
authors.forename: A
authors.initials: A
authors.lastname: Street
authors.forename: J
authors.initials: J
authors.lastname: Lloyd
authors.forename: K
authors.initials: K
authors.lastname: Marsden
mesh_terms: D019411:Clinical Laboratory Techniques / Q000592:standards*; D006801:Humans; D015588:Observer Variation; D011237:Predictive Value of Tests; D011786:Quality Control; D011787:Quality of Health Care; D014842:von Willebrand Diseases / Q000175:diagnosis*