Patients with acquired von Willebrand syndrome (AvWS) who are scheduled for major surgery require perioperative haemostatic intervention. The deficiency in functional von Willebrand factor substantially increases surgical bleeding risk, and restoring adequate haemostatic capacity before, during, and after the procedure is the primary clinical priority.
In the setting of major surgery, vWF/FVIII concentrate therapy is the preferred haemostatic intervention, with postoperative monitoring of haemostatic parameters central to managing the response. Adjunctive therapy may be incorporated depending on the characteristics of the surgical site.
For patients whose AvWS is driven by specific underlying pathology, additional immunomodulatory options may be considered when a more sustained haemostatic effect is needed in advance of a planned procedure.
DOI: 10.5603/ahp.107038
In more complex cases or major surgery, vWF/FVIII concentrates are preferred, with dosages of 30–100 vWF:RCo units/kg and frequent monitoring of FVIII:C in the postoperative period, as it best reflects hemostatic effect.
Antifibrinolytic agents (e.g., tranexamic acid 20–25 mg/kg q8–12h) are recommended as adjunctive therapy, particularly in surgical sites with high fibrinolytic activity, such as oral or gastrointestinal mucosa.
For patients with underlying autoantibodies or paraproteins, IVIG (1 g/kg per 2 consecutive days or 0.4 g/kg per 5 consecutive days) may provide sustained elevation of both vWF and FVIII for several weeks, useful in planned surgical settings.
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