A diagnosis of von Willebrand disease despite normal test results for factor VIII and von Willebrand factor antigen and activity

Motum, Penelope; Just, Sarah; Zebeljan, Diane; Nicholls, Catherine; Kershaw, Geoffrey; Oliver, Susan; Mohammed, Soma; Favaloro, Emmanuel J.

doi:10.1002/ajh.25618

Cited by 4 publications

(2 citation statements)

References 46 publications

(81 reference statements)

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“…In these patients, VWF GPIb-binding assay/Ag ratios (i.e., VWF:RCo/Ag, VWF:GPIbR/Ag, and VWF: GPIbM/Ag) will generally be normal but VWF:CB/Ag ratios will be low. 37,38 The fact that VWF GPIb-binding assay/Ag ratios are normal will again exclude a HMW VWF deficiency (and thus 2A or 2B VWD)-providing the VWF GPIb-binding assay(s) in place is sensitive to such HMW VWF loss. A third group of 2M reflects patients with VWF mutations affecting both CB and GPIb binding.…”

Section: Use Of Assay Panels and Assay Ratiosmentioning

confidence: 99%

“…As another example, we recently reported on a rare 2M VWD with normal VWF test results by all VWF test classes (VWF:Ag, VWF:RCo, and VWF: CB by ELISA, VWF:GPIbR, and VWF:GPIbM) that was only identified because abnormally low VWF:CB/Ag ratios were identified using a new VWF:CB assay based on CLIA. 38 Also relevant is that VWF and FVIII:C represent acute-phase proteins, and may be elevated in individuals due to concurrent illness, or stress. 40 Thus, VWF and FVIII:C may return normal test results in a patient with mild type 1 VWD, and thus mask their VWD.…”

Section: Additional Strategies For Diagnosing Vwdmentioning

confidence: 99%

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Navigating the Myriad of von Willebrand Factor Assays

Favaloro

2020

Hamostaseologie

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Abstractvon Willebrand factor (VWF) represents a large and complex adhesive plasma protein whose main function is to provide a bridge between blood platelets and damaged endothelium, and thus facilitate primary hemostasis. VWF also binds to FVIII, preventing early proteolysis, and delivers this cargo to sites of vascular injury, thereby promoting clot formation and secondary hemostasis. An absence, deficiency, or defect in VWF can lead to a bleeding diathesis called von Willebrand disease (VWD), considered the most common inherited bleeding disorder. Contemporary laboratory assays used in VWD diagnosis/exclusion comprise a myriad of assays that identify the quantity (level) of VWF, as well as the multitude of VWF activities. These may use the following test abbreviations: VWF:Ag, VWF:RCo, VWF:CB, VWF:GPIbR, VWF:GPIbM, VWF:FVIIB, VWF:Ab. The current review explains what these assays are, as well as their place in VWD diagnostics.

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Section: Use Of Assay Panels and Assay Ratiosmentioning

confidence: 99%

Section: Additional Strategies For Diagnosing Vwdmentioning

confidence: 99%