Bleeding‐related hospitalization in patients with von Willebrand disease and the impact of prophylaxis: Results from national registers in Sweden compared with normal controls and participants in the von Willebrand Disease Prophylaxis Network

Holm, Elena; Carlsson, Katarina Steen; Lövdahl, Susanna; Lail, Alice; Abshire, Thomas C.; Berntorp, Erik

doi:10.1111/hae.13473

Cited by 28 publications

(56 citation statements)

References 9 publications

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“…Gastrointestinal (GI) bleeding is the most frequent cause of hospitalization in von Willebrand disease (VWD). 1,2 VWD patients often present with recurrent overt or occult GI bleeding from an unidentified source on conventional endoscopy. 3 This clinical picture is assumed to be related to an increased incidence of GI angiodysplasia in VWD.…”

Section: Introductionmentioning

confidence: 99%

Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

Paris¹,

Repessé

Branche³

et al. 2021

Journal of Thrombosis and Haemostasis

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Background Despite a high prevalence of angiodysplasia, no specific guidelines are available for the modalities of endoscopic exploration of gastrointestinal (GI) bleeding in von Willebrand disease (VWD). Whether VWD patients could benefit from video capsule endoscopy (VCE) looking for angiodysplasia eligible to endoscopic treatment or at high risk of bleeding is unknown. Objectives To assess the diagnostic efficacy for angiodysplasia and the prognostic value of VCE on top of conventional endoscopy in VWD patients with GI bleeding. Patients/Methods A survey was sent to the 30 centers of the French‐network on inherited bleeding disorders to identify VWD patients referred for endoscopic exploration of GI bleeding from January 2015 to December 2017. Data obtained included patient characteristics, VWD phenotype/genotype, GI bleeding pattern, results of endoscopic investigations, and medical management applied including endoscopic therapy. We assessed by Kaplan‐Meier analysis the recurrence‐free survival after the first GI bleeding event according to endoscopic categorization and, in patients with angiodysplasia, to the presence of small‐bowel localizations on VCE exploration. Results GI bleeding source localization was significantly improved when including VCE exploration (P < .01), even in patients without history of angiodysplasia (P < .05). Patients with angiodysplasia had more GI bleeding recurrences (P < .01). A lower recurrence‐free survival was observed in patients with angiodysplasia (log‐rank test, P = .02), and especially when lesions were located in the small bowel (log‐rank test, P < .01), even after endoscopic treatment with argon plasma coagulation (log‐rank test, P < .01). Conclusion VCE should be more systematically used in VWD patients with unexplained or recurrent GI bleeding looking for angiodysplasia eligible to endoscopic treatment or at high risk of relapse.

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Section: Introductionmentioning

confidence: 99%

Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

Paris¹,

Repessé

Branche³

et al. 2021

Journal of Thrombosis and Haemostasis

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“…Few previous studies have reported estimates of VWD prevalence in the general population with estimates ranging between 0.1% and 1%, [8][9][10] and to our knowledge, no previous study has presented results for overall or trends in incidence of VWD at a population level. Thus, while VWD might be the most common bleeding disorder, we lack information about the epidemiology of VWD and the potential unmet treatment needs in this patient population.…”

mentioning

confidence: 83%

“…4 Patients with VWD may experience lower quality of life 6,7 and have more hospital contacts and longer hospital stays compared to the general population. 8 It is therefore important to identify and treat symptomatic VWD early to alleviate the psychological and physiological distress of the individual and the burden on the health care system caused by undiagnosed patients.…”

mentioning

confidence: 99%

Incidence of von Willebrand disease in Denmark, 1995‐2016: A cohort study

et al. 2021

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disease (VWD) is characterized by either partial or complete deficiency (type 1 and type 3) or dysfunction (type 2) of the protein von Willebrand factor. 1 Type 1 accounts for 60%-85% of confirmed cases, while most remaining cases are type 2. 2,3 Type 3 is rare. 4 Among patients diagnosed with VWD, there is a broad spectrum of disease severity. Type 1 is generally a mild to moderate form of the disease, with few or minor bleeding episodes. Type 3 comprises the largest proportion of patients that report bleeding

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“…Long-term prophylaxis in VWD is being used increasingly common 53 to prevent secondary complications, reduce hospitalization, and improve quality of life. 34,54,55 Due to the late onset of clinically relevant bleeding, prophylaxis in patients with VWD is usually secondary prophylaxis, 35,53 which is started in early adulthood. 54 Bleeding scores can help to assess the severity of bleeding tendency.…”

Section: Vwd and Prophylaxismentioning

confidence: 99%

“…34,54,55 Due to the late onset of clinically relevant bleeding, prophylaxis in patients with VWD is usually secondary prophylaxis, 35,53 which is started in early adulthood. 54 Bleeding scores can help to assess the severity of bleeding tendency. 23,56 Indications for prophylaxis may be severe VWD with recurrent forms of GI bleeding, joint bleeding, severe nose bleeding, menorrhagia and in cases which could not adequately controlled with other therapeutic options.…”

Section: Vwd and Prophylaxismentioning

confidence: 99%

Replacement Therapy in Patients with Von Willebrand Disease—Indications and Monitoring

Nowak‐Göttl¹,

Miesbach

Koscielny

et al. 2019

Hamostaseologie

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In patients with von Willebrand disease (VWD), replacement therapy may be indicated in the case of spontaneous bleeding, surgical interventions and injuries/trauma or as a prophylaxis of spontaneous bleeding episodes. The deficient von Willebrand factor (VWF) is replaced with or without factor VIII (FVIII). Dual VWF/FVIII concentrates can be beneficial in the case of low FVIII level, while repeated dosing may lead to very high FVIII levels, with a potential thrombogenic effect in individual VWD patients. An excessive FVIII:C increase can be limited by using a VWF product with a low level of FVIII, achieving a haemostatic adequate FVIII:C increase after 6 to 12 hours. Replacement therapy in patients with VWD shall be individualised considering VWD type, history and risk of bleeding and risk of thrombosis, as well as indication and the individually variable VWF and FVIII increase. Deviations from the dosages and minimum trough levels mentioned in guidelines or recommendations can be considered in justified cases. The objective of this review is to provide recommendations for specific constellations of replacement therapy based on the VWD-specific guidelines available in Europe, the available evidence, own experiences and the consensus of the interdisciplinary German author group.

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Bleeding‐related hospitalization in patients with von Willebrand disease and the impact of prophylaxis: Results from national registers in Sweden compared with normal controls and participants in the von Willebrand Disease Prophylaxis Network

Abstract: Our study indicates that subjects with VWD have a considerably higher consumption of healthcare resources compared to controls and that initiation of prophylaxis may reduce the number of hospitalizations due to bleeding.

Cited by 28 publications

References 9 publications

Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

Incidence of von Willebrand disease in Denmark, 1995‐2016: A cohort study

Replacement Therapy in Patients with Von Willebrand Disease—Indications and Monitoring

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