Clinical haematology · UKMLA & AKT
Von Willebrand disease
A free high-yield preview for the UKMLA Applied Knowledge Test. Below are the key points to recognise von willebrand disease — the full SA Note notes add investigations, management, complications and 10 practice questions.
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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