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Von Willebrand disease

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2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Major bleeding in von Willebrand disease

Intracranial symptoms, haemodynamic instability, major gastrointestinal or postpartum bleeding, expanding neck or airway bleeding and uncontrolled postoperative haemorrhage require immediate factor-supported haemostasis.

Action: Use ABCDE and the major-haemorrhage pathway, contact the haemophilia comprehensive care centre immediately, obtain FBC, coagulation, fibrinogen and VWF or FVIII samples without delaying treatment, control the anatomical source, and give the patient's established VWF-containing concentrate plan rather than an untested empirical desmopressin dose.

Synopsis

Recognise inherited von Willebrand disease, confirm its subtype with correctly timed haemostasis testing, and plan bleeding, procedures, menstruation and pregnancy using desmopressin or VWF replacement safely.

  • VWD is the commonest inherited bleeding disorder and usually causes epistaxis, easy bruising, heavy menstrual bleeding and immediate or delayed procedural haemorrhage.
  • A normal PT and often normal APTT do not exclude VWD; begin with a standardised bleeding history, FBC and ferritin, VWF antigen, platelet-dependent VWF activity and factor VIII.
  • Confirm a borderline result with repeat baseline testing because acute stress, bleeding, pregnancy, oestrogen and inflammation can temporarily raise VWF and mask deficiency.

Key red flags

Head injury, new severe headache, neurological deficit or reduced consciousness in a person with severe VWD requires urgent imaging and haemostatic treatment before deterioration.

Investigation priorities

01
First-line: bleeding assessment, FBC and ferritinFirst stepFirst line

Establish phenotype, inherited pattern and consequences before interpreting VWF numbers.

02
First-line: VWF antigen, VWF activity and FVIIIFirst line

Measure VWF quantity, platelet-dependent function and carrier effect on factor VIII.

Management branches

Suspected inherited VWDCombine phenotype with baseline tests

Mucocutaneous or procedural bleeding, heavy periods or a compatible family history raises suspicion.

  1. Document bleeding across dental, surgical, menstrual and obstetric challenges and construct a three-generation family history where possible.
  2. Send FBC, ferritin, PT, APTT, VWF antigen, platelet-dependent activity and FVIII when the patient is clinically well if feasible.

Key medicines

DesmopressinFor a documented responsive phenotype, give 0.3 micrograms/kg intravenously diluted and infused over 20–30 minutes before the haemostatic challenge; repeat only under the haemophilia plan because response wanes and water retention accumulates.
Tranexamic acidUse 1 g orally three times daily for adult oral, nasal, dental or menstrual bleeding for the shortest effective course, or the exact intravenous or topical regimen in the specialist procedure plan; reduce in renal impairment.
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom