Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management

Siragusa, Sergio; Malato, A.; Coco, Lucio Lo; Cigna, Valentina; Saccullo, Giorgia; Abbene, Ignazio; Anastasio, Raffaela; Caramazza, Domenica; Patti, Rosalia; Arcara, M; Vita, Gaetano Di

doi:10.1111/j.1365-2516.2007.01596.x

Cited by 9 publications

(10 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In their 1993 survey, Fressinaud and Meyer [3] did not identify angiodysplasia in any patients with type 1 VWD whilst we found GI bleeding in nine of 48 patients which was confirmed to be due to angiodysplasia in four individuals. Angiodysplasia in type 1 VWD was also reported in three patients from Italy that are not included in our cohort [10]. In view of the demonstration that VWF has antiangiogenic properties and likely to be aetiological in the development of angiodysplasia, there is no reason why it would not be seen in type 1 disease, and is consistent with the more frequent angiodysplasia in patients with type 3 VWD of 4.5% [2,3,7].…”

Section: Discussionsupporting

confidence: 76%

The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease

et al. 2014

View full text Add to dashboard Cite

Recurrent gastrointestinal bleeding is one of the most challenging complications encountered in the management of patients with von Willebrand disease (VWD). The commonest cause is angiodysplasia, but often no cause is identified due to the difficulty in making the diagnosis. The optimal treatment to prevent recurrences remains unknown. We performed a retrospective study of VWD patients with occult or angiodysplastic bleeding within the setting of the von Willebrand Disease Prophylaxis Network (VWD PN) to describe diagnostic and treatment strategies. Centres participating in the VWD PN recruited subjects under their care with a history of congenital VWD and gastrointestinal (GI) bleeding due to angiodysplasia, or cases in which the cause was not identified despite investigation. Patients with acquired von Willebrand syndrome or those for whom the GI bleeding was due to another cause were excluded. Forty-eight patients from 18 centres in 10 countries were recruited. Seven individuals had a family history of GI bleeding and all VWD types except 2N were represented. Angiodysplasia was confirmed in 38%, with video capsule endoscopy and GI tract endoscopies being the most common methods of making the diagnosis. Recurrent GI bleeding in VWD is associated with significant morbidity and required hospital admission on up to 30 occasions. Patients were treated with multiple pharmacological agents with prophylactic von Willebrand factor concentrate being the most efficient in preventing recurrence of the GI bleeding. The diagnosis and treatment of recurrent GI bleeding in congenital VWD remains challenging and is associated with significant morbidity. Prophylactic treatment with von Willebrand factor concentrate was the most effective method of preventing recurrent bleeding but its efficacy remains to be confirmed in a prospective study.

show abstract

Section: Discussionsupporting

confidence: 76%

The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease

et al. 2014

View full text Add to dashboard Cite

show abstract

“…For the same reasons surgical resection is not useful, even when the actually bleeding area is identified by endoscopic videocapsules (Fig ). The somastatin analogue octreotide, the antifibrinolytic amino acid tranexamic acid and desmopressin are usually of little help to stop bleeding refractory to replacement therapy (Chey et al , ; Bowers et al , ; Siragusa et al , ). When aneamia is severe (below 90 g/l), red blood cell transfusion is needed, whereas iron administration is helpful only for less severe chronic anemia.…”

Section: Angiodysplasia and Congenital Von Willebrand Diseasementioning

confidence: 99%

Von Willebrand disease‐associated angiodysplasia: a few answers, still many questions

Franchini

Mannucci

2013

Br J Haematol

View full text Add to dashboard Cite

Summary The association between angiodysplasia and von Willebrand disease (VWD) has been known for more than 40 years. Bleeding in the gastrointestinal tract associated with angiodysplasia worsens the clinical course of this inherited haemorrhagic disorder and management may become difficult and challenging. Angiodysplasia associated with acquired defects or dysfunctions of von Willebrand factor (VWF) has also been reported in a variety of conditions such as monoclonal gammopathies, Heyde syndrome and in carriers of ventricular assist devices. The most recent advances concerning the mechanistic, clinical and therapeutic aspects of VWD‐associated angiodysplasia are summarized in this review, together with the limitations of our knowledge that warrant further research in the frame of international cooperation.

show abstract

“…Women with VWD have an increased incidence of endometriosis, uterine fibroids and menorrhaghia which, in type 3 VWD, almost always causes iron deficiency and requires long‐term oral contraceptive use. Mucosal vascular abnormalities are also associated with VWD [110,111].…”

mentioning

confidence: 99%

A review of long‐term prophylaxis in the rare inherited coagulation factor deficiencies

Todd

Perry

2010

Haemophilia

View full text Add to dashboard Cite

The rare inherited coagulation factor deficiencies (deficiencies of factors I, II, V, VII, XI, XIII, combined FV + FVII deficiency, combined deficiency of the vitamin K dependent factors and von Willebrand disease type 3) have an aggregate prevalence of approximately 1:100,000. They may cause recurrent life or function threatening haemorrhage. In this article we review the available literature on long-term prophylaxis and, where possible, make recommendations on this important area.

show abstract

Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management

Cited by 9 publications

References 5 publications

The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease

The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease

Von Willebrand disease‐associated angiodysplasia: a few answers, still many questions

A review of long‐term prophylaxis in the rare inherited coagulation factor deficiencies

Contact Info

Product

Resources

About