Practical aspects of factor concentrate use in patients with von Willebrand disease undergoing invasive procedures: a European survey

Windyga, Jerzy; Dolan, G.; Altisent, Carmen; Κατσαρού, Όλγα; Fernández, María Fernanda López; Zülfikar, Bulent

doi:10.1111/hae.12955

Cited by 15 publications

(22 citation statements)

References 35 publications

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“…The main treatment options for the perioperative management of VWD patients are DDAVP and replacement factor concentrates. European Haemophilia Treatment Standardisation Board (EHTSB) recommendations for the practical use of DDAVP and factor concentrates in VWD patients undergoing invasive procedures have recently been published. The suitability of DDAVP should be determined by a test infusion in candidate patients .…”

Section: Treatment Of Vwdmentioning

confidence: 99%

“…Factor concentrates (VWF/FVIII or VWF) are the treatment of choice in patients in whom DDAVP is ineffective or contraindicated . EHTSB recommendations on the perioperative dosing and monitoring of factor concentrates in VWD patients undergoing surgery, together with other published guidelines/recommendations, are summarized in Table .…”

Section: Treatment Of Vwdmentioning

confidence: 99%

“…EHTSB recommendations on the perioperative dosing and monitoring of factor concentrates in VWD patients undergoing surgery, together with other published guidelines/recommendations, are summarized in Table . In the most recent publication, the EHTSB highlighted a number of important issues . For example, the ratio of VWF:RCo/FVIII:C in the concentrates varies significantly and doses of concentrate expressed in IU dL −1 are not interchangeable.…”

Section: Treatment Of Vwdmentioning

confidence: 99%

“…Therefore, the recommended dose of a given concentrate should be specific to that concentrate as the required dose can be completely different when another concentrate is used. Similarly, because various types of VWD are characterized by great variability in VWF:RCo and FVIII:C, patients should have baseline values of VWF:RCo and FVIII:C measured before undergoing major surgery to plan treatment . For monitoring, FVIII:C seems to be more important than VWF:RCo .…”

Section: Treatment Of Vwdmentioning

confidence: 99%

“…Similarly, because various types of VWD are characterized by great variability in VWF:RCo and FVIII:C, patients should have baseline values of VWF:RCo and FVIII:C measured before undergoing major surgery to plan treatment . For monitoring, FVIII:C seems to be more important than VWF:RCo . The parallel decay of VWF:RCo and FVIII:C curves with Wilate ® (Figure ), a VWF/FVIII with VWF:RCo/FVIII:C ratio of 1:1, may facilitate dosing and monitoring via either VWF:RCo or FVIII:C, while differing half‐lives for VWF:RCo and FVIII:C in other concentrates might require monitoring of both factor levels.…”

Section: Treatment Of Vwdmentioning

confidence: 99%

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Fifth Åland Island conference on von Willebrand disease

et al. 2018

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The fifth Åland Island meeting on von Willebrand disease (VWD) was held on the Åland Islands, Finland, from 22 to 24 September 2016-90 years after the first case of VWD was diagnosed in a patient from the Åland Islands in 1926. This meeting brought together experts in the field of VWD to share knowledge and expertise on current trends and challenges in VWD. Topics included the storage and release of von Willebrand factor (VWF), epidemiology and diagnostics in VWD, treatment of VWD, angiogenesis and VWF inhibitors.

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Section: Treatment Of Vwdmentioning

confidence: 99%

Section: Treatment Of Vwdmentioning

confidence: 99%

Section: Treatment Of Vwdmentioning

confidence: 99%

Section: Treatment Of Vwdmentioning

confidence: 99%

Section: Treatment Of Vwdmentioning

confidence: 99%

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Fifth Åland Island conference on von Willebrand disease

et al. 2018

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Current and Emerging Options for the Management of Inherited von Willebrand Disease

Heijdra

Cnossen

Leebeek

2017

Drugs

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Von Willebrand disease (VWD) is the most common inherited bleeding disorder with an estimated prevalence of ~1% and clinically relevant bleeding symptoms in approximately 1:10,000 individuals. VWD is caused by a deficiency and/or defect of von Willebrand factor (VWF). The most common symptoms are mucocutaneous bleeding, hematomas, and bleeding after trauma or surgery. For decades, treatment to prevent or treat bleeding has consisted of desmopressin in milder cases and of replacement therapy with plasma-derived concentrates containing VWF and Factor VIII (FVIII) in more severe cases. Both are usually combined with supportive therapy, e.g. antifibrinolytic agents, and maximal hemostatic measures. Several developments such as the first recombinant VWF concentrate, which has been recently licensed for VWD, will make a more “personalized” approach to VWD management possible. As research on new treatment strategies for established therapies, such as population pharmacokinetic-guided dosing of clotting factor concentrates, and novel treatment modalities such as aptamers and gene therapy are ongoing, it is likely that the horizon to tailor therapy to the individual patients’ needs will be extended, thus, further improving the already high standard of care in VWD in most high-resource countries.

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