Evidence map›Paper›PMID 34429677›Full record

ReviewJournal of blood medicine2021

Why is Misdiagnosis of von Willebrand Disease Still Prevalent and How Can We Overcome It? A Focus on Clinical Considerations and Recommendations.

Chanukya K Colonne, Benjamin Reardon, Jennifer Curnow, Emmanuel J Favaloro

Abstract readReview
In one paragraph

Review in Journal of blood medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

23 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Trial
  3. Article
  4. Review
  5. Review
  6. Von Willebrand disease diagnosis: from complexity to simplicity.Research and practice in thrombosis and haemostasis · 2025
    Article
  7. Acquired von Willebrand syndrome in children.Research and practice in thrombosis and haemostasis · 2025
    Article
  8. Review
  9. Article
  10. Article
  11. Article
  12. Review
  13. To Clot or Not to Clot: Is That the Question?Journal of clinical medicine · 2023
    Article
  14. Laboratory-based inequity in thrombosis and hemostasis: review of the evidence.Research and practice in thrombosis and haemostasis · 2023
    Review
  15. Article
  16. Post-analytical Issues in Hemostasis and Thrombosis Testing: An Update.Methods in molecular biology (Clifton, N.J.) · 2023
    Article
  17. Article
  18. An Overview of Laboratory Testing for ADAMTS13.Methods in molecular biology (Clifton, N.J.) · 2023
    Article
  19. Review
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

4 authors.

Chanukya K Colonne *Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Sydney, NSW, Australia.
Benjamin Reardon *Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Sydney, NSW, Australia.
Jennifer CurnowDepartment of Clinical Haematology, Westmead Hospital, Sydney, NSW, Australia.ORCID 0000-0002-0569-2873
Emmanuel J FavaloroDepartment of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Sydney, NSW, Australia.ORCID 0000-0002-2103-1661

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Despite von Willebrand disease (VWD) being the most common inherited bleeding disorder, its accurate diagnosis is frequently shrouded by diagnostic pitfalls. VWD is frequently under-diagnosed, over-diagnosed and misdiagnosed, leading to significant avoidable patient morbidity and health care system burden. At the heart of this dilemma lies the heterogeneity and complexity of von Willebrand factor (VWF) and associated defects, and the necessity of coalescing clinical and laboratory features to obtain an accurate diagnosis. Common pitfalls include poor clinical and scientific understanding and familiarity with VWD, incomplete clinical history and lack of routine use of standardised bleeding assessment tools (BAT), difficulty in accessing a comprehensive repertoire of laboratory tests, significant pre-analytical, analytical and post-analytical issues, and lack of expertise in laboratory testing and interpretation. Errors, resulting in under-diagnosis, over-diagnosis, and misdiagnosis of VWD, are presented and discussed. Strategies to minimise errors include better education of clinicians and laboratory staff on VWD, routine use of validated BAT, utilising a comprehensive gamut of laboratory investigations according to a standardised algorithm, and repeating testing to minimise pre-analytical errors. Recommendations on appropriate patient selection for VWD testing, how VWD should be investigated in the laboratory, and how to ensure test results are accurately interpreted in the correct clinical context are detailed.

Indexed as

diagnosisvon Willebrand diseaseVWD

Identifiers

PMID34429677
PMCPMC8380198

What OpenQuestion holds

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LicenceCC BY-NC
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.