Von Willebrand's Disease - Type 1
- 3 types based on severity of bleeding disorder:
- lower than normal levels of vWF - most common
- structural defect → ↓vWF activity (multiple types)
- very little or no vWF
→ beeding into muscles/joints ± spontaneous
- ** most common bleeding diathesis in Australia
- ≈ 1% of the world's population vWD+
- ≈ 90% people with vWD have not been diagnosed
- ≈ 200,000 people have vWD in Australia
(National Blood Authority - Australian Bleeding Disorders 2023-24)
- Australian Bleeding Disorders Registry
Management:
- DDAVP → 2-3x ↑VIII:vWF
- Type 1, occasionally in Type 2
- never effective in Type 3 vWD!
- Antifibrinolytics (Tranexamic acid)
- mucosal bleeding (mouth, epistaxis, menorrhagia)
- adjunct therapy to DDAVP / factor concentrate
- vWF/FVIII Plasma Concentrate - Biostate®
- human plasma-derived product
- vial: 50 IU/mL FVIII and 100 IU/mL vWF
- bleeding with Type 2 and Type 3 vWD
- rarely in Type 1 vWD if severe unresponsive bleeding
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