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. 2020 Jul 15;4(6):952-957.
doi: 10.1002/rth2.12392. eCollection 2020 Aug.

Classification of von Willebrand disease in the context of modern contemporary von Willebrand factor testing methodologies

Emmanuel J Favaloro  1   2   3
Affiliations

Classification of von Willebrand disease in the context of modern contemporary von Willebrand factor testing methodologies

Emmanuel J Favaloro. Res Pract Thromb Haemost. .
No abstract available

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Figures

FIGURE 1
FIGURE 1
F (A) Algorithm that describes a diagnostic process for VWD that takes into account the differential utility of different VWF:CB methods, as well as VWF multimers, from the author’s experience and perspective. Ag, antigen; CB, collagen binding; FVIII, factor VIIII; GPIb, glycoprotein Ib (platelet VWF receptor); GPIbM, GPIb mutation‐based assay; GPIbR, recombinant GPIb–based assay; HMW, high‐molecular‐weight (VWF); LIA, latex immunoassay; N, normal; NA, not applicable; PFA, platelet function analyzer; RCo, ristocetin cofactor; RIPA, ristocetin‐induced platelet aggregation; VWD, von Willebrand disease; VWF, von Willebrand factor. (B) Summary of ranges for percentage of low‐molecular‐weight multimers (LMWMs), intermediate‐molecular‐weight multimers (IMWMs), and high‐molecular‐weight multimers (HMWMs), for different sample groupings for previously published studies with comparable sample numbers. 15 , 18 , 23 Of interest, the publications from Bowyer et al 23 and Favaloro et al 18 seem to more closely align with one another, showing more overlaps between VWD groups (ie, less definitive discrimination) than findings reported by Vangenechten and Gadisseur. 15 This is likely to be reflective of test sample cohorts, with those of Vangenechten and Gadisseur 15 reflecting well‐characterized genetically confirmed cases from a VWD biobank. In contrast, cases from the prior publications reflect those arising in “real‐world diagnostic test practice.” In other words, most laboratories applying the methodology to diagnostics are unlikely to achieve the clear separations reported by Vangenechten and Gadisseur 15 (Note: SHP=standard human plasma [=pooled normal plasma])
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References

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