Laboratory diagnosis of von Willebrand disease in the age of the new guidelines: considerations based on geography and resources

Favaloro, Emmanuel J.; Pasalic, Leonardo

doi:10.1016/j.rpth.2023.102143

Cited by 10 publications

(4 citation statements)

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“…The detailed description of the methods used for assessing the specific VWF activities recommended in IGL have been reported previously in another review article 27 and more recently have been updated by Favaloro and Pasalic. 28 In their review article, Favaloro and Pasalic pointed out the advantages of the PD-VWFact versus the old VWF:RCo and discussed the potency of the automated assays commercially available that were previously described. 29 30 31 32 33 34 Favaloro and Pasalic agreed with IGL members who recommended to avoid the old VWF:RCo assay and to use the most recent PD-VWFact tests.…”

Section: Tests Of Von Willebrand Factor Activities Useful For Diagnos...mentioning

confidence: 99%

“…13 The methods and role of the other laboratory assays useful in the VWD diagnosis have been described in detail previously. 27 28 The procoagulant activity of factor VIII (FVIII:C) is usually very low in VWD3 patients, who are also characterized by undetectable levels of VWF, while FVIII:C is normal in most cases with VWD2A, VWD2B, and VWD2M. The FVIII:C/VWF:Ag ratio is a useful parameter for the identification of VWD types because values >1 suggest VWD1 and <1 VWD2N.…”

Section: Tests Of Von Willebrand Factor Activities Useful For Diagnos...mentioning

confidence: 99%

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Current Diagnosis of von Willebrand Disease in Italy: 3 Years Following the Release of the International Guidelines

Federici

2024

Semin Thromb Hemost

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The American Society of Hematology–International Society on Thrombosis and Haemostasis–National Hemophilia Foundation–World Federation of Hemophilia 2021 International Guidelines (IGL) on von Willebrand disease (VWD) have pointed out many challenges, mainly in the diagnostic approach of VWD patients. To determine the impact of these IGL on the current clinical and laboratory diagnosis of Italian VWD patients, we have recently conducted a survey among 43 centers affiliated with the Italian Association of Hemophilia Centers (AICE). Directors and colleagues responsible for the management of VWD patients were invited to report in a detailed questionnaire how IGL recommendations about the assessment of the specific activities of von Willebrand Factor (VWF) could be applied at their local sites. Results from such a survey showed that bleeding assessment tools, VWF antigen, and factor VIII procoagulant are currently in use in all centers. The automated assays for platelet-dependent VWF activity with or without ristocetin described in IGL have been used since 2021 in 37/43 (86%) centers. Among other laboratory tests, VWF collagen binding, ristocetin-induced platelet agglutination, multimeric analysis, VWF propeptide, VWF:FVIII binding assay were available in 49, 63, 26, 7, and 28% of AICE, respectively. Analyses of VWF gene defects are available only at 3/43 (7%) centers. Desmopressin (DDAVP) infusion trials at diagnosis, with measurements of VWF activities at 1 and 4 hours post-DDAVP, is currently performed at 38/43 (88%) centers. Based on this information, a simplified clinical diagnosis using a few automated tests before and after DDAVP has been proposed. Such a diagnostic approach will be validated prospectively in a large cohort of Italian VWD patients.

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Section: Tests Of Von Willebrand Factor Activities Useful For Diagnos...mentioning

confidence: 99%

Section: Tests Of Von Willebrand Factor Activities Useful For Diagnos...mentioning

confidence: 99%

Current Diagnosis of von Willebrand Disease in Italy: 3 Years Following the Release of the International Guidelines

Federici

2024

Semin Thromb Hemost

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“…Thus, it seems to be the assay most frequently used in place of the VWF:RCo assay, and is recommended by recently issued guidelines. 1,9 Other common methods measure VWF collagen-binding activity (VWF:CB), or VWF-factor VIII binding (VWF:FVIIIB) capacity. Where the multimeric composition of VWF is of interest, qualitative electrophoretic methods are available to separate and visualise VWF multimers.…”

Section: Introductionmentioning

confidence: 99%

“…Many clinicians are not aware that it is a VWF:GpIb binding assay, but assume it is similar to the traditional VWF:RCo assay. Thus, it seems to be the assay most frequently used in place of the VWF:RCo assay, and is recommended by recently issued guidelines 1,9 . Other common methods measure VWF collagen‐binding activity (VWF:CB), or VWF‐factor VIII binding (VWF:FVIIIB) capacity.…”

Section: Introductionmentioning

confidence: 99%

In vitro field study and worldwide survey assessing how clinical haemostasis laboratories analyse recombinant and plasma‐derived von Willebrand factor products

Turecek,

Ilk,

Gritsch

2023

Haemophilia

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IntroductionSeveral well‐established clinical laboratory methods are available to measure von Willebrand factor (VWF) in plasma samples, but few data are available on their use for analysing recombinant VWF (rVWF).AimTo evaluate how clinical diagnostic laboratories analyse rVWF and plasma‐derived VWF (pdVWF) spiked in vitro into VWF‐deficient plasma using quantitative protein and functional assays of VWF.MethodsHuman VWF‐deficient plasma samples were spiked with rVWF (vonicog alfa; Takeda) or pdVWF/factor VIII (pdVWF/FVIII; antihemophilic factor/VWF complex [human], CSL Behring), each at final concentrations of 1.0, 0.6, 0.2, 0.1 IU/mL VWF:ristocetin cofactor activity (VWF:RCo) according to labelled VWF activity. The ISTH SSC secondary coagulation standard was used as a control. Participating laboratories received three sets of these blinded aliquots. Mean results per assay were compared with the expected potency based on the labelled VWF:RCo activity.ResultsAmong 39 laboratories, the most commonly established assay was VWF:RCo; 22 laboratories reported data from 2214 tests. Despite a trend to lower values, VWF:RCo activities for rVWF were in agreement with target concentrations (71%–109%), whereas VWF:platelet glycoprotein Ib (VWF:GpIb) and VWF collagen‐binding activity (VWF:CB) assays gave high recoveries (up to 132% and 127%, respectively). In contrast, pdVWF/FVIII was substantially underestimated by VWF:GpIb and VWF:CB assays (56%–86% recoveries), whereas the VWF:RCo assay gave recoveries of 47%–112% for pdVWF/FVIII.ConclusionThe results of VWF assays used in clinical laboratories differ between rVWF and pdVWF, particularly for VWF:GpIb and VWF:CB assays. These differences may arise from the higher multimeric structure of rVWF compared to pdVWF.

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Misleading antigenic von Willebrand factor levels in acquired von Willebrand syndrome secondary to monoclonal gammopathy of undetermined significance

Okamoto,

Suzuki,

Tamura

et al. 2024

Int J Hematol

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Laboratory diagnosis of von Willebrand disease in the age of the new guidelines: considerations based on geography and resources

Cited by 10 publications

References 49 publications

Current Diagnosis of von Willebrand Disease in Italy: 3 Years Following the Release of the International Guidelines

Current Diagnosis of von Willebrand Disease in Italy: 3 Years Following the Release of the International Guidelines

In vitro field study and worldwide survey assessing how clinical haemostasis laboratories analyse recombinant and plasma‐derived von Willebrand factor products

Misleading antigenic von Willebrand factor levels in acquired von Willebrand syndrome secondary to monoclonal gammopathy of undetermined significance

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