Clinical haematology · UKMLA & AKT
Von Willebrand disease
A free high-yield preview for the UKMLA Applied Knowledge Test, covering how to recognise this condition. The full SA Note notes add investigations, management and complications, with practice questions on this topic.
Key high-yield points
- Most common heritable bleeding disorder - affects males and females equally (distinguishes from haemophilia)
- Caused by deficiency or dysfunction of von Willebrand factor (VWF) - a large multimeric glycoprotein essential for primary haemostasis
- Type 1 accounts for ~70-80% of cases; Type 3 is rarest and most severe
- VWF has two roles: (1) platelet adhesion bridge - collagen → VWF → GPIb on platelets; (2) carrier protein for Factor VIII (FVIII), protecting it from degradation
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