Overview
Definition
Most common inherited bleeding disorder, affecting platelet adhesion.
Epidemiology
Affects up to 1% of the population.
Etiology & Risk Factors
- Autosomal dominant quantitative or qualitative defect in von Willebrand Factor (vWF)
Clinical Symptoms
- Mucocutaneous bleeding (epistaxis, easy bruising)
- Heavy menstrual bleeding (menorrhagia)
Clinical Approach
Diagnosis
- Prolonged bleeding time
- Normal or slightly prolonged aPTT
- Low Ristocetin cofactor assay
Management
- Desmopressin (DDAVP)
- vWF/Factor VIII concentrates
- Tranexamic acid (antifibrinolytic)
Complications
- Severe bleeding after dental extraction or surgery