Key clinical practice guidelines and consensus documents for the diagnosis, laboratory evaluation, and management of von Willebrand disease
Why this library matters
Clinical practice guidelines differ in scope, methodology, and purpose.
Some focus on diagnosis.
Others address treatment, laboratory evaluation, or practical implementation.
Some provide comprehensive overviews, whereas others answer only a limited number of carefully selected clinical questions.
No single document answers every question about von Willebrand disease.
This library is intended to help clinicians quickly identify which guideline is most useful for a particular clinical problem, while providing direct access to the original documents.
For an overview of how to read, interpret, and apply clinical practice guidelines, see:
➡️ Clinical Practice Guidelines in von Willebrand Disease: Where Evidence Ends and Judgment Begins
At a glance
| Guideline | Year | Primary focus | Best starting point for… |
|---|---|---|---|
| NHLBI Expert Panel Report | 2008 | Diagnosis & management | Comprehensive overview and historical foundation |
| Nordic Guidance | 2011 | Practical implementation | Specialist-center care and practical management |
| UKHCDO Guideline | 2014 | Diagnosis & management | Understanding Low VWF and UK diagnostic philosophy |
| ASH/ISTH/NHF/WFH Diagnosis Guideline | 2021 | Diagnosis | Diagnostic thresholds, BATs, assay selection |
| ASH/ISTH/NHF/WFH Management Guideline | 2021 | Management | Surgery, pregnancy, heavy menstrual bleeding, prophylaxis |
| UKHCDO/BSH Laboratory Guideline | 2024 | Laboratory diagnosis | Laboratory interpretation, assay selection, genetics |
NHLBI Expert Panel Report (2008)
🟪 Historical Foundation
Primary focus
Comprehensive diagnosis and management of inherited and acquired von Willebrand disease.
Best used for
Understanding the conceptual framework that shaped modern VWD diagnosis and management.
Covers
- Initial evaluation
- Laboratory diagnosis
- VWD classification
- Desmopressin
- VWF replacement
- Surgery
- Pregnancy
- Acquired VWS
Why this document still matters
Although many laboratory recommendations have evolved, this report established the diagnostic framework that still underlies contemporary VWD practice.
Main limitation
Predates GRADE methodology, newer platelet-dependent activity assays, and contemporary approaches to genetic interpretation.
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Nordic Guidance (2011)
🟦 Practical Implementation
Primary focus
Practical diagnosis and management within Nordic hemophilia centers.
Best used for
Understanding how VWD care can be organized within a specialist bleeding-disorders program.
Covers
- Repeat testing
- DDAVP assessment
- Surgery
- Pregnancy
- Longitudinal follow-up
Why this document still matters
Provides an excellent example of translating guideline recommendations into day-to-day clinical practice.
Main limitation
Reflects regional practice and predates more recent international recommendations.
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UKHCDO Guideline (2014)
🟨 Diagnosis & Management
Primary focus
Diagnosis and management of inherited VWD.
Best used for
Understanding the practical distinction between Low VWF and classic type 1 VWD.
Covers
- Diagnostic approach
- Laboratory evaluation
- Low VWF
- DDAVP
- Surgery
- Pregnancy
- Replacement therapy
Why this document still matters
This guideline gave the Low VWF concept practical clinical prominence and continues to influence international discussions regarding diagnostic thresholds.
Main limitation
Predates the 2021 international guidelines and the 2024 laboratory guidance.
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ASH/ISTH/NHF/WFH Diagnosis Guideline (2021)
🟩 Diagnosis
Primary focus
Evidence-based diagnosis of VWD using GRADE methodology.
Best used for
Current diagnostic thresholds and evidence-based laboratory evaluation.
Covers
- Bleeding assessment tools
- Diagnostic thresholds
- Platelet-dependent activity assays
- Activity-to-antigen ratios
- Age-related normalization
- Type 1C
- Multimers
- Collagen binding
- Type 2 testing
- Genetic testing
Why this document still matters
This is the current international reference for evidence-based diagnosis and diagnostic decision-making.
Main limitation
Addresses selected diagnostic questions rather than every aspect of diagnosis.
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ASH/ISTH/NHF/WFH Management Guideline (2021)
🟥 Management
Primary focus
Evidence-based management of VWD.
Best used for
Procedural management and selected therapeutic decisions.
Covers
- Long-term prophylaxis
- Desmopressin trials
- Major surgery
- Minor procedures
- Heavy menstrual bleeding
- Pregnancy
- Neuraxial anesthesia
- Postpartum tranexamic acid
- Antithrombotic therapy
Why this document still matters
Provides transparent, evidence-based recommendations for many of the most common management questions encountered in clinical practice.
Main limitation
Addresses selected management questions rather than functioning as a comprehensive treatment textbook.
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Joint UKHCDO/BSH Laboratory Guideline (2024)
🟧 Laboratory Diagnosis
Primary focus
Laboratory diagnosis and monitoring of VWD.
Best used for
Selecting, performing, and interpreting laboratory investigations.
Covers
- Preanalytical variables
- Activity assays
- Activity-to-antigen ratios
- Collagen binding
- Multimers
- FVIII-binding assays
- RIPA
- Genetics
- Acquired VWS
- Monitoring
Why this document still matters
The most comprehensive contemporary resource for laboratory evaluation and interpretation in VWD.
Main limitation
Focused almost entirely on laboratory diagnosis and monitoring rather than broader clinical management.
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Which guideline should I use?
| Clinical question | Recommended starting document |
|---|---|
| Is this patient likely to have VWD? | 2021 International Diagnosis Guideline |
| Which laboratory assays should I order or trust? | 2024 UKHCDO/BSH Laboratory Guideline |
| How should I evaluate a patient with Low VWF? | UKHCDO Guideline (2014) together with the 2021 Diagnosis Guideline |
| How should I manage surgery? | 2021 International Management Guideline |
| How should I manage pregnancy or delivery? | 2021 International Management Guideline |
| How should I evaluate assay discordance? | 2024 UKHCDO/BSH Laboratory Guideline |
| I want a broad overview of VWD. | NHLBI Expert Panel Report (2008) |
| I want practical implementation advice. | Nordic Guidance (2011) |