Evidence map›Paper›PMID 33570651›Full record

ArticleBlood advances2021

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

Paula D James, Nathan T Connell, Barbara Ameer, Jorge Di Paola, Jeroen Eikenboom, Nicolas Giraud, Sandra Haberichter, Vicki Jacobs-Pratt, Barbara Konkle, Claire McLintock and 9 more

3 registry-linked trialsOpen access · goldAbstract read
In one paragraph

Article in Blood advances, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 3 registered trials, which are not on this map. Cited by 244 papers, 7 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
244citing papers in PubMed, 7 pooled it
50.9field-weighted citation impact, top 1% of its field
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.

NCT05116501 nacompletednot on this mapstarted 2022, after this paper: background citation

Novel Insights Into the Genetic Background of Patients With Low Von Willebrand Factor Levels Using Next-generation Sequencing

TypeinterventionalSponsorFondazione IRCCS Ca' Granda, Ospedale Maggiore PoliclinicoRan2022 to 2025Enrolled254ConditionsLow Von Willebrand FactorArmsWhole-exome sequencing
NCT05500807 phase1recruitingnot on this mapstarted 2022, after this paper: background citation

Emicizumab for Severe VON Willebrand Disease (VWD) and VWD/Hemophilia A

TypeinterventionalSponsorBleeding and Clotting Disorders Institute Peoria, IllinoisRan2022 to 2028Enrolled40ConditionsVon Willebrand Disease, Type 3, Concomitant VWD and HemophiliaArmsEmicizumab
NCT06736158 early_phase1recruitingnot on this mapstarted 2025, after this paper: background citation

Early Genomic Testing for Inherited Bleeding Disorders in Patients Without a Diagnosis After First Line Testing: a Randomized Controlled Trial

TypeinterventionalSponsorQueen's UniversityRan2025 to 2027Enrolled212ConditionsBleeding DisorderArmsGenetic testing for inherited bleeding disorders
3 · Its place in the literature

Who cites it

244 citing papers in PubMed, 7 syntheses or guidelines pooled it, 508 citations in OpenAlex.

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  17. Seventh Åland Island Meeting on von Willebrand Disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2026
    Article
  18. Article
  19. Article
  20. von Willebrand disease: an illustrated review.Research and practice in thrombosis and haemostasis · 2026
    Review

184 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

19 authors at 14 institutions in 6 countries.

Paula D JamesDepartment of Medicine, Queen's University, Kingston, ON, Canada.
Nathan T ConnellBrigham and Women's Hospital, Harvard Medical School, Boston, MA.
Barbara AmeerPharmacology Consulting, Princeton Junction, NJ.
Jorge Di PaolaDepartment of Pediatrics, Washington University in St. Louis, St. Louis, MO.
Jeroen EikenboomDivision of Thrombosis and Hemostasis, Department of Internal Medicine, Leiden University Medical Center, Leiden, The Netherlands.
Nicolas GiraudMarseille, France.
Sandra HaberichterDiagnostic Laboratories, Versiti Blood Research Institute, Milwaukee, WI.
Vicki Jacobs-PrattAuburn, ME.
Barbara KonkleBloodworks Northwest, Seattle, WA.
Claire McLintockNational Women's Health, Auckland City Hospital, Auckland, New Zealand.
Simon McRaeNorthern Cancer Service, Launceston General Hospital, Launceston, TAS, Australia.
Robert R MontgomeryVersiti Blood Research Institute, Milwaukee, WI.
James S O'DonnellIrish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Nikole ScappeCoraopolis, PA.
Robert SidonioAflac Cancer and Blood Disorders, Children's Healthcare of Atlanta, Emory University, Atlanta, GA.
Veronica H FloodVersiti Blood Research Institute, Milwaukee, WI.
Nedaa HusainatOutcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS.
Mohamad A KalotOutcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS.
Reem A MustafaOutcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS.
University of Kansas Medical Center · USVersiti Blood Center of Wisconsin · USAuburn University · USAuckland City Hospital · NZBrigham and Women's Hospital · USEmory University · USLaunceston General Hospital · AULeiden University Medical Center · NLMedical College of Wisconsin · USQueen's University · CARoyal College of Surgeons in Ireland · IERutgers, The State University of New Jersey · USUniversity of Washington · USWashington University in St. Louis · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundvon Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges.

objectiveThese 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.

methodsASH, 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.

resultsThe panel agreed on 11 recommendations.

conclusionsKey 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.

Indexed as

Hemophilia AThrombosisvon Willebrand DiseasesHemorrhageHemostasisHumans

Identifiers

PMID33570651
PMCPMC7805340
OpenAlexW3121114429

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

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.