Von Willebrand disease – the ‘Dos’ and ‘Don'ts’ in surgery

Abstract

Von Willebrand disease (VWD) is the most common genetic bleeding disorder. VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilises coagulation factor VIII (FVIII) in the blood. Prophylaxis of surgical bleeding in patients with VWD requires consideration of the individual situation, including the type of procedure and the bleeding rate, before decisions about treatment type, dose, duration and adjunctive therapy with antifibrinolytics or antithrombotic prophylaxis can be made. Although desmopressin (DDAVP)-stimulated release of endogenous VWD is an effective treatment strategy in many patients, plasma concentrates containing VWF are the preferred option for most patients undergoing surgical procedures. Recommendations for the management of surgery in patients with VWD are summarised, including the severity of VWD and the type of the surgical procedure.

Perioperative bleeding and thrombotic risks in patients with Von Willebrand disease

“…[7,8] Although current measures to mitigate surgical bleeding in WVD appear to be safe and well tolerated in small series, effects of pharmacology on perioperative thrombotic risks remain uncertain. [7–9]…”

Laboratory monitoring of replacement therapy for major surgery in von Willebrand disease

“…A key issue in the management of major surgical procedures in VWD is the optimal dosing of replacement therapy and the laboratory monitoring of dose–response relationship . Pertaining to dosage and infusion frequencies several guidelines or expert panels have offered recommendations (Table ).…”

Replacement therapy during surgery in von Willebrand disease needs personalization

“…We do not know how thrombogenic procedures were implemented or the nature of the risk factors among those subjects not receiving thromboprophylaxis. We recently published a paper in which the “Dos and Don'ts” in surgery in the presence of VWD were discussed, and important issues for consideration were listed . The relevance of these issues is underscored by the paper by Hazendonk and colleagues.…”

“…This is not as “easy” as it is in haemophilia, where population PK models have been published and are implemented in clinical practice . The complex variation between VWD concentrate brands and VWD types makes population PK and personalized care more difficult to develop in VWD and, so far, prediction of levels during surgery using parameters such as blood group or pharmacokinetics does not seem reliable . This is, consequently, a field of clinical research with a high priority for patients, health care and society.…”

“…We recently published a paper in which the "Dos and Don'ts" in surgery in the presence of VWD were discussed, and important issues for consideration were listed. 10 The relevance of these issues is underscored by the paper by Hazendonk and colleagues. A take-home message from these papers, as well as other reports of surgery in patients with VWD, may well be: plan surgery at experienced centres having close collaboration between the surgery and haemophilia departments and be sure that you know the characteristics of your patient (VWD type, factor levels, thrombotic risk), the concentrate (VWF/FVIII ratio, pharmacokinetic properties, as these may differ between brands 7 ) and the surgical procedure (minor, major, risks of thrombosis or bleeding).…”