### Introduction von Willebrand disease (vWD) is the most common inherited bleeding disorder. Although perioperative management should be coordinated with hematology, HPB surgeons should understand the major subtypes and broad principles of treatment when planning major operations. Core Concept von Willebrand factor (vWF) has two essential functions: 1. Primary hemostasis: Mediates platelet adhesion by binding exposed subendothelial collagen to platelet GPIb receptors. 2. Secondary hemostasis: Serves as the carrier protein for factor VIII, protecting it from degradation. As a result, vWD causes both impaired platelet function and varying degrees of factor VIII deficiency. ### Types Type 1 – “Too Little” - Partial quantitative deficiency of vWF - Accounts for ~75–80% of cases - Usually mild - Treatment: DDAVP if responsive Type 2 – “Doesn’t Work” Qualitative defects in vWF function. Type 2A – Missing Large Multimers - Loss of the highest-molecular-weight multimers responsible for effective platelet adhesion - Treatment: Usually vWF concentrate for major bleeding or surgery Type 2B – Binds Platelets Too Well - Gain-of-function mutation causing spontaneous platelet binding and thrombocytopenia - Treatment: vWF concentrate - Avoid DDAVP Type 2M – Defective Platelet Binding - Multimers are present but function abnormally - Treatment: Similar to Type 2A Type 2N – Defective Factor VIII Binding - Impaired binding and stabilization of factor VIII - Can resemble mild hemophilia A - Treatment: vWF replacement Type 3 – “Almost None” - Near-complete absence of vWF - Severe bleeding phenotype - Treatment: vWF concentrate - DDAVP is ineffective ### Treatment #### Desmopressin (DDAVP) Mechanism - Stimulates endothelial release of endogenous vWF and factor VIII. Indications - First-line for most patients with Type 1. - May be effective in selected patients with Type 2A or 2M after demonstrating an adequate response. Avoid - Type 2B - Type 3 (ineffective) Dose - IV: 0.3 mcg/kg over 20–30 minutes (maximum 20–30 mcg) - Intranasal: 150 mcg (<50 kg) or 300 mcg (≥50 kg) Key adverse effect - Hyponatremia—restrict free water for approximately 24 hours following administration. #### von Willebrand Factor Concentrates Examples: - Humate-P - Wilate - Vonvendi Indications - Major surgery - Severe bleeding - Type 2B - Type 3 - DDAVP nonresponders Typical dosing - Loading: 40–60 IU/kg preoperatively - Maintenance: 20–40 IU/kg every 12–24 hours, adjusted to vWF/FVIII levels and clinical bleeding - Continue for 1–5 days after minor procedures and 7–14 days after major surgery #### Tranexamic Acid Useful adjunct for mucosal bleeding and peri-procedural hemostasis. Dose - 1 g PO or IV every 8 hours - Typically continued for 3–5 days, depending on bleeding risk Key Points - vWF mediates platelet adhesion and stabilizes factor VIII. - Type 1 is the most common; Type 3 is the most severe. - Type 2B is associated with thrombocytopenia and DDAVP should be avoided. - DDAVP is first-line therapy for most patients with Type 1. - Major HPB operations often require vWF concentrate replacement with perioperative hematology guidance and serial monitoring of vWF and factor VIII levels. Last updated: 5 August 2026