Comparison of an automated chemiluminescent assay to a manual ELISA assay for determination of von Willebrand Factor collagen binding activity on VWD plasma patients previously diagnosed through molecular analysis of <i>VWF</i>

Jousselme, Emilie; Jourdy, Yohann; Rugeri, Lucia; Négrier, Claude; Nougier, Christophe

doi:10.1111/ijlh.12743

Cited by 13 publications

(8 citation statements)

References 30 publications

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“…Ten addressed diagnostic test accuracy for types 2A and 2B 59,85-93 and 6 for type 2M. [87][88][89][90]92,93 For types 2A and 2B, the sensitivity of VWF:CB/VWF:Ag was 0.90 (95% CI, 0.78-0.96) and specificity was 0.95 (95% CI, 0.89-0.98), vs a sensitivity of 0.90 (95% CI, 0.90-0.99) and specificity of 0.97 (95% CI, 0.94-0.99) for multimers. For type 2M VWD, the sensitivity of VWF:CB/VWF:Ag was 0.98 (95% CI, 0.96-1.00) with a specificity of 0.99 (95% CI, 0.98-1.00), vs a sensitivity of 0.86 (95% CI, 0.73-0.98) and a specificity of 0.97 (95% CI, 0.94-0.99) for multimers.…”

Section: Remarkmentioning

confidence: 99%

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

et al. 2021

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Background: von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges. Objective: These evidence-based guidelines of the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) are intended to support patients, clinicians, and other health care professionals in their decisions about VWD diagnosis. Methods: ASH, ISTH, NHF, and WFH established a multidisciplinary guideline panel that included 4 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Outcomes and Implementation Research Unit at the University of Kansas Medical Center (KUMC) supported the guideline-development process, including performing or updating systematic evidence reviews up to 8 January 2020. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subsequently subject to public comment. Results: The panel agreed on 11 recommendations. Conclusions: Key recommendations of these guidelines include the role of bleeding-assessment tools in the assessment of patients suspected of VWD, diagnostic assays and laboratory cutoffs for type 1 and type 2 VWD, how to approach a type 1 VWD patient with normalized levels over time, and the role of genetic testing vs phenotypic assays for types 2B and 2N. Future critical research priorities are also identified.

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Section: Remarkmentioning

confidence: 99%

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

et al. 2021

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“…VWF:CB is usually tested by an ELISA but recently an automated and rapid chemiluminescence method has become available. 61 Typically, a mixture of type I or type III collagen is used because it increases specificity. 62 The A3 VWF domain binds type I and type III collagen, while the A1 domain binds type IV and type VI collagen, but assays testing this latter interaction are not widely available.…”

Section: Laboratory Testing For Vwdmentioning

confidence: 99%

Obstacles to Early Diagnosis and Treatment of Inherited von Willebrand Disease: Current Perspectives

Castaman

Linari

2021

JBM

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Von Willebrand disease (VWD), the most common inherited bleeding disorder, is highly heterogeneous, and its early diagnosis may be difficult, especially for mild cases and in qualitative von Willebrand factor (VWF) defects. Appropriate VWD diagnosis requires the combination of personal and/or family history of bleeding and abnormal VWF laboratory testing. The use of bleeding assessment tools has been helpful in standardizing bleeding history collection and quantification of bleeding symptoms to select patients who may benefit of further hemostatic testing. Type 1 and 3 VWD which represent quantitative VWD variants are relatively easy to diagnose. The diagnosis of type 2 VWD requires multiple assessments to evaluate the effects induced by the responsible abnormality on the heterogeneous functions of VWF. Sensitive and reproducible tests are needed to evaluate different VWF activities, starting from measuring VWF-platelet interaction. In the recent years, several increasingly sensitive, rapid and automated assays have been developed, but they are not widely available so far. Genetic testing for VWD diagnosis is not a common practice because VWF gene is very large and highly polymorphic and therefore it is used only in specific cases. It is evident that the early and correct VWD diagnosis allows optimal management of bleeding and situations at risk. Tranexamic acid, desmopressin, replacement therapy with plasmaderived concentrates with a variable content of VWF and FVIII, or the new recombinant VWF are the different therapeutic options available. Careful VWD classification guides treatment because desmopressin is widely used in type 1 while replacement therapy is the cornerstone of treatment for type 2 and 3 variants.

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“…7 The collagen binding assay (VWF:CB) reveals the adhesion capacity of HMW multimers of VWF to collagen. 8 This assay is useful to reduce the misleading diagnosis of VWD and should be widely adopted in hematology laboratories. Favaloro et al showed that limiting the assays to FVIII:C, VWF:Ag, and VWF:RCo can induce a high background diagnostic error rate (approximately 20% error rate for type 1 VWD misidentified as type 2 VWD, approximately 30% for type 2 VWD misidentified as type 1 VWD, and nearly 5% for normal samples being identified as being from VWD patients).…”

Section: Vwd Is Associated With Genetic Defects In the Vonmentioning

confidence: 99%

“…Additionally, analysis of VWF:RCo/VWF:Ag is performed to predict quantitative (ratio ≥0.6) and qualitative (ratio <0.6) VWF deficiencies . The collagen binding assay (VWF:CB) reveals the adhesion capacity of HMW multimers of VWF to collagen . This assay is useful to reduce the misleading diagnosis of VWD and should be widely adopted in hematology laboratories.…”

Section: Introductionmentioning

confidence: 99%

Standardization and comparison of nonautomated assays to measure the collagen binding activity of von Willebrand factor

Oliveira

Amorim

Corsini

et al. 2018

Int J Lab Hematology

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Comparison of an automated chemiluminescent assay to a manual ELISA assay for determination of von Willebrand Factor collagen binding activity on VWD plasma patients previously diagnosed through molecular analysis of VWF

Abstract: Introduction: The correct diagnosis and classification of VWD (von Willebrand dis-

Cited by 13 publications

References 30 publications

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

Obstacles to Early Diagnosis and Treatment of Inherited von Willebrand Disease: Current Perspectives

Standardization and comparison of nonautomated assays to measure the collagen binding activity of von Willebrand factor

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