Performance evaluation and multicentre study of a von Willebrand factor activity assay based on GPIb binding in the absence of ristocetin

Patzke, Juergen; Budde, Ulrich; Huber, Adrian Thomas; Méndez, Adriana; Muth, Heidrun; Obser, Tobias; Peerschke, Ellinor I.B.; Wilkens, Matthias; Schneppenheim, Reinhard

doi:10.1097/mbc.0000000000000169

Cited by 67 publications

(62 citation statements)

References 13 publications

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“…16 There is reasonable correlation between VWF:RCo and VWF:GPIbM results. 15 One study did show increased qualitative VWF defects using the VWF:GPIbM. 16 This may be due to use of ristocetin as the "gold standard," when in reality ristocetin is not the most accurate assay.…”

Section: Vwf and Gpibamentioning

confidence: 99%

“…The VWF:GPIbM assay, using the terminology recommended by the International Society on Thrombosis and Haemostasis, introduces gain-of-function mutations into GPIba, allowing it to bind VWF spontaneously in vitro without the requirement for ristocetin. 15 The VWF:GPIbM allows greater precision, with a reported lower limit of detection of 2 IU/dL and a reported-within-laboratory coefficient of variation of 5.6%. 16 There is reasonable correlation between VWF:RCo and VWF:GPIbM results.…”

Section: Vwf and Gpibamentioning

confidence: 99%

“…At the time of writing, commercial availability of VWF:GPIbM assays is limited, but in some countries these have replaced the VWF:RCo entirely. In Europe and Canada, an automated VWF:GPIbM is available, 15,16 whereas in the US, an ELISA version is available through the BloodCenter of Wisconsin. 17 Both use a combination of 2 gain-of-function GPIba variants to elicit binding in the absence of ristocetin.…”

Section: Vwf and Gpibamentioning

confidence: 99%

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Advances in the diagnosis and treatment of Von Willebrand disease

Sharma

Flood

2017

Blood

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Von Willebrand disease (VWD) is the most common inherited bleeding disorder, yet diagnosis and management remain challenging. Development and use of bleeding assessment tools allows for improved stratification of which patients may require further assessment and which patients are most likely to require treatment of their VWD. New options for laboratory assessment of von Willebrand factor (VWF) activity include a new platelet-binding assay, the VWF:GPIbM, which is subject to less variability than the ristocetin cofactor activity assay, and collagen-binding assays that provide insight into a different function of VWF. Genetic testing may be helpful in some cases where a type 2 VWD variant is suspected but is usually not helpful in type 1 VWD. Finally, treatment options for VWD are reviewed, including the use of recombinant VWF. Despite these advances, still more work is required to improve diagnosis, treatment, and quality of life for affected patients.

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Section: Vwf and Gpibamentioning

confidence: 99%

Section: Vwf and Gpibamentioning

confidence: 99%

Section: Vwf and Gpibamentioning

confidence: 99%

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Advances in the diagnosis and treatment of Von Willebrand disease

Sharma

Flood

2017

Blood

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“…[41][42][43][44] More recently, automatic VWF:GPIb-binding assays independent of ristocetin have been developed and introduced into general use with promising results. [45][46] …”

Section: Additional Automatic and Novel Assays For Phenotypic Diagnmentioning

confidence: 99%

Clinical and laboratory diagnosis of VWD

Federici

2014

Hematology

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VWD is the most common inherited bleeding disorder and is due to a deficiency and/or abnormality of VWF. VWD is inherited in an autosomal-dominant or autosomal-recessive pattern, but women are apparently more symptomatic. Three main criteria are required for correct diagnoses of VWD: (1) positive bleeding history since childhood, (2) reduced VWF activity in plasma, and (3) history of bleeding in the family. The bleeding score, together with baseline VWF levels and family history, have been proposed as more evidence-based criteria for VWD. Measurements of a reduced VWF activity in plasma are essential for the diagnosis of VWD; assays for the evaluation of the interactions between VWF and platelet glycoprotein Ib receptor with or without ristocetin, as well as VWF collagen binding, are currently in use. However, other tests such as VWF antigen, factor VIII, ristocetin-induced platelet agglutination, multimeric analysis, VWF propeptide, VWF/FVIII binding assay, and assessment of biological response to desmopressin are necessary to characterize VWD types. Levels of VWF activities Ͻ30 U/dL have been associated with a bleeding phenotype and the presence of mutations in the VWF gene. Learning Objectives• To identify patients at risk for VWD according to their history of bleeding using a specific questionnaire to calculate BS • To use appropriate laboratory tests to classify VWD patients • To predict the bleeding phenotype of VWD and the need for replacement therapy using BS and baseline VWF levels

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“…10,13,14 Additionally, Patzke et al have described performance of the INNOVANCE VWF Ac (Siemens, Marburg, Germany) on the BCS XP System (Siemens) and the Sysmex CA-7000, CA-1500, CS-2000i, and CA-560 analyzers, which are also based on GPIb binding in the absence of ristocetin. 15 Ristocetin-less assays, now collectively termed VWF:GPIbM, 16 are steps toward improving the laboratory diagnosis of VWD. Additionally, clinically relevant VWF activity properties are being uncovered as described in collagen-VWF interactions.…”

Section: Introductionmentioning

confidence: 99%