Mucosal bleeding, ↑aPTT, ↑bleeding time, normal platelets
Confirm: VWF antigen + ristocetin cofactor
Minor / type 1 → Desmopressin (DDAVP) 0.3 µg/kg (releases stored VWF) — contraindicated in type 2B; tranexamic acid useful adjunct for mucosal bleeding/menorrhagia
Major → VWF / Factor VIII concentrate
Order set
- VWF antigen + activity
- Factor VIII
- FBC, coags
- Blood group (type O lower VWF)
Criteria
AdmitSignificant bleeding
TransfuseVWF/FVIII concentrate for major bleed
DischargeBleeding controlled, prophylaxis/plan for procedures
Clinical detail — differentials, red flags, pitfalls, disposition
Red flags
- Significant procedural/menstrual bleeding
Differentials
- Haemophilia
- Platelet function disorder
Common mistakes
- Relying on bleeding time
- Missing acquired VWD
Disposition & follow-up
DDAVP trial; VWF concentrate for major bleeds; haematology.
Discharge package
MedicationsDDAVP/VWF plan for bleeds/procedures
Follow-upHaematology; pre-procedure planning
Warning symptomsProlonged/heavy bleeding
💊 Treatment detail — doses & preparation
DDAVP (haemostasis)VWF releaser
Dose0.3 mcg/kg IV (max 20 mcg) over 30 min — type 1 VWD/mild haemophilia A
PreparationDilute in 50 mL NS; test-dose response before reliance
MonitorNa⁺, fluid restriction 24 h; tachyphylaxis with repeated doses
Factor VIII concentratefactor replacement
DoseDose = weight × desired % rise × 0.5; major bleed target 100% then 50% ×3–7 d
PreparationReconstitute vial with supplied diluent; slow IV push
MonitorFactor level post-dose; inhibitor screen if no response
Tranexamic acidantifibrinolytic
Dose1 g IV/PO TDS (bleeding in VWD/haemophilia adjunct, menorrhagia)
Preparation500 mg tablets; IV 1 g in 50 mL NS over 10 min
MonitorAvoid with PCC/combined hormonal contraception (thrombosis); haematuria caution (clot colic)
📖 ASH-ISTH-NHF-WFH VWDReviewed July 2026