von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)<sup>1</sup>

Nichols, William L.; Hultin, Mae B.; James, Andra H.; Manco‐Johnson, M.; Montgomery, Robert R.; Ortel, Thomas L.; Rick, Margaret E.; Sadler, J. Evan; Weinstein, Mark J.; Yawn, Barbara P.

doi:10.1111/j.1365-2516.2007.01643.x

Cited by 840 publications

(1,206 citation statements)

References 404 publications

(717 reference statements)

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“…Others consider any detectable VWF to represent type 1 VWD, albeit a more severe phenotype, and reserve the label of type 3 VWD for those with completely undetectable VWF levels 2, 7. The distinction is relevant to the inheritance, as type 1 is considered to be autosomal dominant and type 3 autosomal recessive, but not to treatment, as any patient with VWF levels <10 IU/dL will likely require treatment with a VWF concentrate.…”

Section: Classification Of Von Willebrand Diseasementioning

confidence: 99%

“…These patients will have a decreased VWF:Ag, a low VWF:GPIbM/VWF:Ag ratio, a low VWF:CB/VWF:Ag ratio, and an absence of HMWM. Platelet binding (VWF:PB) and low‐dose ristocetin‐induced platelet aggregation (LD‐RIPA) on these patients would be absent 7. Genetic testing may reveal a known type 2A variant and help solidify the diagnosis.…”

Section: Classification Of Von Willebrand Diseasementioning

confidence: 99%

“…The former NHLBI consensus guideline statement used a cut‐off of 30 IU/dL to declare the presence of VWD 7. UK guidelines have also adopted this cutoff 24.…”

Section: Diagnosis Of Vwdmentioning

confidence: 99%

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Current issues in diagnosis and treatment of von Willebrand disease

Keesler

Flood

2018

Research and Practice in Thrombosis and Haemostasis

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Essentials Classification of VWD includes quantitative (types 1 and 3) and qualitative (type 2) variants.New assays of VWF‐platelet binding may improve accuracy in diagnosis.Collagen binding represents an additional function of VWF not measured by current tests.Treatment options for VWD include desmopressin, plasma‐derived, and recombinant VWF. Clinically, von Willebrand disease (VWD) presents as mucosal bleeding caused by a decreased quantity or quality of von Willebrand factor (VWF). Diagnosis of VWD requires careful consideration of patient specific factors, bleeding symptoms, and laboratory results. Patients with borderline low VWF levels remain challenging, given that low VWF is not necessarily a guarantee of bleeding, but is present in many patients with symptoms, and treatment of low VWF may improve bleeding. Laboratory diagnosis of VWD is complex and no single test can determine the presence or absence of functional VWF. Historically, VWF binding to platelet GPIbα was measured by the ristocetin cofactor assay (VWF:RCo); a new assay using platelet GPIbα in the absence of ristocetin (VWF:GPIbM) is gradually replacing the VWF:RCo due to improved accuracy in diagnosis. VWF binding to collagen is a separate function, and requires specific testing to determine if a collagen binding defect is present. Regardless of these laboratory complexities, clinicians can empirically treat VWD to alleviate bleeding symptoms by raising VWF levels through desmopressin or VWF concentrate. Recombinant VWF is now available, but clinicians may need to add an initial dose of FVIII when treating emergency bleeds.

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Section: Classification Of Von Willebrand Diseasementioning

confidence: 99%

Section: Classification Of Von Willebrand Diseasementioning

confidence: 99%

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Current issues in diagnosis and treatment of von Willebrand disease

Keesler

Flood

2018

Research and Practice in Thrombosis and Haemostasis

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“…As it was not designed to relate hemostatic traits and the bleeding questionnaire to the outcome bleeding, the perioperative significance of (mild) hemostatic abnormalities and the diagnostic value of a positive bleeding questionnaire regarding the identification of patients who bleed during surgery remain unknown. However, to optimize perioperative preventive measures, establishing a diagnosis of a hemostatic deficiency is preferred over recognizing a patient as having a “positive bleeding history,” as measures to prevent bleeding differ per disorder 35, 36, 37. Other limitations concern the study design that led to an imbalance between the two patient groups and the relative low number of hemostatic abnormalities that was found.…”

Section: Discussionmentioning

confidence: 99%

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Vries

Meijden

Kuiper

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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Essentials Patients with bleeding disorders are at risk of operative bleeding, but screening for these disorders is challenging.Patients with and without bleeding symptoms on a guideline‐based screening questionnaire were included and hemostatically phenotyped.The questionnaire could not differentiate between patients with and without hemostatic abnormalities.The discriminative power of the PT, aPTT, TT, Euglobulin lysis time, PFA, and the ISTH‐BAT was also limited. BackgroundPatients with mild bleeding disorders are at risk of perioperative bleeding, but screening for these disorders remains challenging.ObjectivesWe aimed to assess the prevalence of hemostatic abnormalities in patients with and without reported bleeding symptoms on a preoperative questionnaire, consisting of guideline‐proposed questions, and appraised the diagnostic value of several screening modalities for the identification of patients with hemostatic abnormalities.MethodsIn this observational study, 240 patients with and 95 patients without bleeding symptoms on the preoperative questionnaire were included. Patients with known bleeding disorders, antithrombotic drugs, thrombocytopenia, and anemia were excluded. Preoperatively, all patients underwent elaborate hemostatic testing. Hemostatic abnormalities were defined as coagulation, vWF, or fibrinolysis factor levels below reference range and platelet function defects. Screening modalities included the ISTH Bleeding Assessment Tool (ISTH‐BAT), PT, aPTT, TT, Euglobulin Lysis Time (ELT), and Platelet Function Analyser (PFA).ResultsIn 21 of 240 (8.8%) patients reporting bleeding symptoms, hemostatic abnormalities were found, including 7 reduced coagulation factor levels, 10 platelet function abnormalities, and 4 reduced vWF levels. In comparison, 10 of 95 (10.5%) patients not reporting bleeding symptoms had abnormalities. The ISTH‐BAT could not identify patients with abnormalities, while PT, aPTT, TT, ELT, and PFA had high specificity but low sensitivity to detect abnormalities.ConclusionsThe prevalence of hemostatic abnormalities in both patients with and without reported bleeding symptoms was 9%‐10%. This suggests that the guideline‐based questionnaire cannot differentiate between patients with and without abnormalities, while the discriminative power of the screening modalities is also limited.

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“…After written informed consent was obtained the patients provided research blood samples. Patients were asked to complete a bleeding questionnaire (Appendix) modified from the consensus conference on the assessment of von Willebrand disease as previously described 2, 10. For patients with positive bleeding questionnaire responses, further chart review and patient history acquisition were performed to identify clinically significant bleeding, and to determine whether Heyde syndrome (transfusion‐dependent anemia resulting from GI bleeding and endoscopically documented angiodysplasia) was present.…”

Section: Methodsmentioning

confidence: 99%

Von Willebrand factor multimer quantitation for assessment of cardiac lesion severity and bleeding risk

Austin

Chen

Thomas

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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Essentials VWF multimers have an established association with valvular heart disease.Significant interlaboratory variation exists in VWF multimeric analysis.We describe a method to normalize VWF multimers for assessment of cardiac lesion severity and clinical bleeding.Normalized VWF multimer ratios improved the diagnostic capabilities of the assay. Backgroundvon Willebrand factor (VWF) multimer quantitation has been utilized in the assessment of valvular heart disease, however, there is no standardized method for quantitation. We compared three methods of assessment which utilized a normal plasma control.MethodsWe analyzed 476 samples and their control plasma from 368 patients with valvular heart disease, hypertrophic cardiomyopathy, or LVAD therapy, and 27 normal subjects. VWF multimers were assessed as normalized VWF multimer ratios (NMR) of gel bands >15/2‐15 (NMR15) or gel bands >10/2‐10 (NMR10). Associations of VWF laboratory and multimeric assessments with cardiac lesion severity and acquired bleeding were investigated.ResultsAbnormal multimers were present in 78% of patients with moderate to severe hemodynamic abnormalities compared to 19% of patients with normal or mildly abnormal hemodynamics. NMR showed strong association with severe cardiac lesions (NMR15: OR 15.29, CI 9.04‐27.18; NMR10: OR 14.18, CI 8.88‐23.21). PFA‐CADP was strongly associated with moderate to severe cardiac lesions (OR 14.91, CI 9.08‐24.50). PFA‐CADP and NMR15 showed excellent ability to discriminate ≥moderate (AUC 0.86, CI 0.83‐0.89 and 0.83, CI 0.79‐0.87 respectively) and severe cardiac lesions (AUC 0.84, CI 0.81‐0.88 and 0.85, CI 0.81‐0.88 respectively). NMR was less strongly associated with bleeding (OR 4.01 for NMR10, CI 2.49‐6.58).ConclusionQuantification of VWF multimers may provide clinical utility in circumstances where clinical estimation of cardiac lesion severity is challenging, such as with dysfunctional prosthetic valves. The presence of abnormal VWF multimers is associated with bleeding, however further quantitation provided only modest improvement in risk stratification.

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von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)¹

Cited by 840 publications

References 404 publications

Current issues in diagnosis and treatment of von Willebrand disease

Current issues in diagnosis and treatment of von Willebrand disease

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Von Willebrand factor multimer quantitation for assessment of cardiac lesion severity and bleeding risk

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von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)1

Cited by 840 publications

References 404 publications

Current issues in diagnosis and treatment of von Willebrand disease

Current issues in diagnosis and treatment of von Willebrand disease

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Von Willebrand factor multimer quantitation for assessment of cardiac lesion severity and bleeding risk

Contact Info

Product

Resources

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von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)¹