Von Willebrand disease and gastrointestinal bleeding: A national inpatient sample study

Tsagianni, Anastasia; Comer, Diane; Yabes, Jonathan G.; Ragni, Margaret V.

doi:10.1016/j.thromres.2019.04.017

Cited by 14 publications

(23 citation statements)

References 25 publications

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“…All patients were explored at least once with conventional endoscopy. Overall, angiodysplasia were identified in 26% of patients after conventional endoscopy: new additional angiodysplasia was identified in 50% of patients with preexisting angiodysplasia and de novo angiodysplasia in 20% of patients without preexisting angiodysplasia, a proportion in line with a recent study also reporting the frequency of angiodysplasia on conventional endoscopy 2 . Besides angiodysplasia, other lesions were identified in 32% of patients after conventional endoscopy (Table 1).…”

Section: Resultssupporting

confidence: 83%

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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: Resultssupporting

confidence: 83%

“…During follow‐up, angiodysplasia were identified endoscopically in 46% of patients confirming angiodysplasia as the leading cause of GI bleeding in VWD 2 . Patients with angiodysplasia were older than patients with obscure GI bleeding ( P = .002) and their most frequent presentation was melena.…”

Section: Resultsmentioning

confidence: 89%

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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show abstract

“…Previous studies have shown that gastrointestinal bleeding almost exclusively occurs in patients that have a reduction of HMW VWF multimers, which is the case in type 2A, type 2B and type 3 VWD (Leebeek & Eikenboom, ). Gastrointestinal bleeding is more common in young, black or male VWD patients (Tsagianni et al , ). The most significant predictors of bleeding include angiodysplasia, diverticulitis, hepatitis C and smoking (Tsagianni et al , ).…”

Section: Management In Specific Situationsmentioning

confidence: 99%

“…Gastrointestinal bleeding is more common in young, black or male VWD patients (Tsagianni et al , ). The most significant predictors of bleeding include angiodysplasia, diverticulitis, hepatitis C and smoking (Tsagianni et al , ).…”

Section: Management In Specific Situationsmentioning

confidence: 99%

“…Many VWD patients have recurrent gastrointestinal bleeding, despite treatment with factor concentrates. This may be caused by angiodysplasia or vascular malformations in the intestines, which give a higher chance for continuation of bleeding or re‐bleeding (Makris et al , ; Tsagianni et al , ). Many drugs have been used to treat VWD patients with persistent gastrointestinal bleeding.…”

Section: Management In Specific Situationsmentioning

confidence: 99%

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How I manage severe von Willebrand disease

Leebeek¹,

Atiq²

2019

Br J Haematol

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Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding. Moreover, we review emerging treatments that may be applied in future management of patients with severe VWD.

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Polidocanol foam sclerotherapy in the treatment of hemorrhoidal disease in patients with bleeding disorders: a multicenter, prospective, cohort study

et al. 2022

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Background The management of hemorrhoidal disease (HD) in patients with bleeding disorders (BD) is challenging. Polidocanol foam sclerotherapy (PFS) is associated with a low rate of bleeding complications. The aim of this study was to compare the efficacy and safety of PFS in the treatment of HD in patients with and without BD. Methods This prospective, multicenter, cohort study enrolled patients with (group B) and without (group A) BD, with symptomatic internal HD grades I–III over an 18-month period. All patients were treated with PFS. Patients with congenital BD did not undergo prior replacement therapy and those with acquired BD due to antithrombotic drugs, did not discontinue therapy. Efficacy outcomes included therapeutic success and HD recurrence during a 1-year follow-up period. To evaluate safety the complications related to PFS were recorded. Results We included 228 patients (group A: 155, group B: 73; male/female: 114/114; mean age: 59.4 ± 15.9 years). The baseline hemorrhoidal disease bleeding grade ( p < 0.001 ) and Sodergren hemorrhoidal symptom severity score ( p = 0.019 ) were higher for group B. The overall therapeutic success rate was 93.4% with an average number of sessions of 1.51 ± 0.74, significantly higher for group B (1.68 ± 0.86 vs 1.43 ± 0.65, p = 0.013 ). Complications occurred in 11.4% of the patients, with bleeding reported in 4.8%. The majority of complications were mild (96.2%). No significant differences between the two groups were observed for therapeutic success, recurrence, or complication rate. Conclusions Patients with BD may have more symptomatic HD at baseline. Even so, PSF showed similar effectiveness and safety in patients with BD compared to patients without BD. Supplementary Information The online version contains supplementary material available at 10.1007/s10151-022-02600-5.

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Von Willebrand disease and gastrointestinal bleeding: A national inpatient sample study

Cited by 14 publications

References 25 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

How I manage severe von Willebrand disease

Polidocanol foam sclerotherapy in the treatment of hemorrhoidal disease in patients with bleeding disorders: a multicenter, prospective, cohort study

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