The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization

Laffan, Michael; Brown, Simon; Collins, Peter William; Cumming, A. M.; Hill, F. G. H.; Keeling, David; Peake, I. R.; Pasi, K. J.

doi:10.1111/j.1365-2516.2004.00894.x

Cited by 158 publications

(183 citation statements)

References 99 publications

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“…All of the other data on the type and frequency of bleeds were as expected from previous retrospective studies. [1][2][3][4][5][6][7][8][9][10]25 DDAVP was used not only in VWD1 but also in more than half of the bleeders with VWD2A and VWD2M, the most intensive regimens being used in VWD2A and VWD1. DDAVP was the only treatment of the majority of minor bleeding events (epistaxis, menorrhagia, gum bleeds), although it had to often be used together with VWF/FVIII concentrates to handle GI bleeding or hematomas.…”

Section: Discussionmentioning

confidence: 99%

“…[6][7][8][9][10] VWD is due to quantitative and/or qualitative defects of von Willebrand factor (VWF), a multimeric glycoprotein synthesized by endothelial cells and megakaryocytes that mediates platelet adhesion/aggregation and stabilizes factor VIII (FVIII) in the circulation. [1][2][3] In VWD, bleeding events are caused not only by impaired platelet-VWF interactions, usually evaluated in plasma by ristocetin cofactor activity (VWF:RCo), but also by reduced FVIII levels that often accompany the VWF defect.…”

Section: Introductionmentioning

confidence: 99%

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The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease

Federici

Bucciarelli

Castaman

et al. 2014

Blood

122

127

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Key Points• The bleeding score helps to predict clinical outcomes in adult patients with von Willebrand disease.• High bleeding scores correlate with intensive ondemand therapy and may identify cases requiring regular prophylaxis.Analyses of the bleeding tendency by means of the bleeding score (BS) have been proposed until now to confirm diagnosis but not to predict clinical outcomes in patients with inherited von Willebrand disease (VWD). We prospectively followed up, for 1 year, 796 Italian patients with different types of VWD to determine whether the previous BS of European VWD1 is useful to predict the occurrence of spontaneous bleeds severe enough to require replacement therapy with desmopressin (DDAVP) and/or von Willebrand factor (VWF)/factor VIII concentrates. Among the 796 patients included, 75 (9.4%) needed treatment of 232 spontaneous bleeding events. BS >10 and VWF:ristocetin cofactor activity <10 U/dL were associated with the risk of bleeding, but only a BS >10 remained highly associated in a multivariable Cox proportional hazard model (adjusted hazard ratio: 7.27 [95% confidence interval, 3.83-13.83]). Although the bleeding event-free survival was different in VWD types, only a BS >10 could predict for each type which patient had bleeding events severe enough to require treatment with DDAVP and/or concentrates. Therefore, BS can be considered a simple predictor of clinical outcomes of VWD and may identify patients needing intensive therapeutic regimens. (Blood. 2014;123(26):4037-4044)

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease

Federici

Bucciarelli

Castaman

et al. 2014

Blood

122

127

View full text Add to dashboard Cite

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“…FVIII level can be reduced in VWD as VWF protects FVIII from proteolytic cleavage [134]. Comprehensive guidelines on the diagnosis of VWD have been produced by the UKHCDO [135].…”

Section: Pt and Activated Pttmentioning

confidence: 99%

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

et al. 2006

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Summary. The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/ gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.

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“…9,10 Tranexamic acid could possibly have caused mentioned adverse effects such as dizziness, nausea, diarrhea and abdominal pain. 11,12 Commercially available recombinant von Willebrand factor-containing concentrates are treatment of choice for most patients with type 2 and type 3 with vWD. Infused together with recombinant factor VIII has shown high efficacy to stop the bleeding without significant side effects.…”

Section: Discussionmentioning

confidence: 99%

Von Willebrand disease as a significant risk factor for hemorrhage after cervical loop electrosurgical excision

Ungure

Žodžika

Kunicina

et al. 2018

Int J Reprod Contracept Obstet Gynecol

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Loop electrosurgical excision procedure (LEEP) is the first-line treatment for cervical intraepithelial neoplasia (CIN). It is effective, easy to perform and relatively safe for the patient. However, 1-2% of patients experience complications such as hemorrhage and infection, which usually are effectively treated using cauterization and antibiotics. We are presenting a case of repeated cervical and vaginal hemorrhage after the cervical LEEP for a patient without previous medical history of severe bleeding or detected bleeding disorders. A generally healthy 38-year-old female underwent planned LEEP of cervix because of CIN II-III. Procedure was successful. Afterwards she was admitted to the Emergency department several times because of recurrent vaginal and cervical bleeding. She received suturing and coagulation of the cervix and vaginal wall combined with desmopressin and tranexamic acid therapy. Because of suspected bleeding disorder hematologist was invited. Laboratory analysis showed positive results for von Willebrand disease. After VIII/von Willebran factor concentrate treatment bleeding was stopped completely. Before any surgical interventions it is important to take a detailed medical history. If atypical, recurrent and/or severe secondary hemorrhage after the surgical manipulations occur, coagulation disorders should be excluded.

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The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization

Cited by 158 publications

References 99 publications

The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease

The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Von Willebrand disease as a significant risk factor for hemorrhage after cervical loop electrosurgical excision

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