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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Von Willebrand disease

Haematology and transfusion · UK scope · Sources checked 27 Aug 2026 · Clinical 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.
  • Subtype is clinically important: compare activity with antigen, assess FVIII and use multimer, collagen-binding, low-dose ristocetin or genetic testing through a specialist laboratory when indicated.
  • Preferred treatment for a responsive mild type 1 phenotype is often desmopressin after a documented test dose; it is ineffective in type 3 and may be unsafe in type 2B.
  • For major bleeding, major surgery, childbirth or non-responsive disease, use a VWF-containing concentrate with product-specific dosing and VWF and FVIII targets from the haemophilia centre.
  • Tranexamic acid is valuable for dental, nasal, oral and menstrual bleeding and is commonly combined with desmopressin or concentrate, but requires renal adjustment and site-specific cautions.
  • Every patient should have a written bleeding plan, haemophilia-centre contact, medicine and procedure advice, and coordinated menstrual, pregnancy and postpartum planning.

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.

Persistent postpartum bleeding can recur after initially adequate haemostasis as pregnancy-elevated VWF falls; obstetric and haemophilia teams must coordinate prevention and follow-up.

Type 2B disease or platelet-type VWD may develop or worsen thrombocytopenia with desmopressin, so subtype and prior trial response must be checked before use.

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.
  3. Repeat borderline or discordant results and refer to a haemophilia centre for subtype testing before definitive treatment decisions.

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.Test response when possible. Avoid in type 3 and generally type 2B, and use caution in cardiovascular disease. Restrict hypotonic fluid and monitor sodium; children, older adults and repeated doses have greater hyponatraemia risk.
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.Review active thrombosis and upper urinary-tract bleeding, where clot obstruction is a concern. Accumulation in severe kidney impairment increases toxicity, and it does not replace factor support for major haemorrhage.
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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.

  • UKHCDO and BSH: laboratory diagnosis and monitoring of von Willebrand diseaseCurrent UK laboratory testing, classification and monitoring guidance.
  • UKHCDO and BSH: diagnosis and management of von Willebrand diseaseUK phenotype, desmopressin, concentrate, surgery and pregnancy principles.
  • BNF desmopressin monographUK dosing, contraindications and fluid and sodium safety.
  • NICE NG24: blood transfusion recommendationsNational component-support and reassessment context during major bleeding.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom