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Physiological AdaptationMost common inherited bleed

Von Willebrand DiseaseNCLEX cheat sheet

Von Willebrand disease is the most common inherited bleeding disorder, caused by a deficiency or defect of von Willebrand factor (vWF), which is needed for platelet adhesion and also carries/stabilizes factor VIII.

27 key facts 3 practice questions Reviewed Oct 2026
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Mechanism

  • ↓vWF → poor platelet adhesion
  • Autosomal, affects both sexes

Signs

  • Mucosal bleeds: epistaxis, gums
  • Menorrhagia, easy bruising
  • Prolonged bleed after surgery

Labs

  • ↑bleeding time, ↓vWF, ±↑PTT

Do

  • DDAVP → ↑vWF release
  • No aspirin/NSAIDs
Red flag to remember

Heavy or uncontrolled bleeding, signs of significant blood loss (tachycardia, hypotension, dizziness) require prompt reporting.

Before you read

Test yourself first: 3 questions

Question 1 of 3

The nurse is assessing a newborn for Hirschsprung disease. Which finding in the first 48 hours of life is most suggestive of this disorder?

What is von willebrand disease?

Unlike hemophilia it is usually autosomal and affects both sexes. Bleeding is typically mucocutaneous: easy bruising, nosebleeds, gum bleeding, and heavy menstrual periods.

Key points to remember

  • vWF mediates platelet adhesion to injured vessel walls and protects factor VIII from breakdown, so its loss impairs primary hemostasis.
  • Most common pattern is mucosal bleeding: epistaxis, gingival bleeding, easy bruising, and menorrhagia in women.
  • Bleeding time and PFA closure time are prolonged; aPTT may be prolonged if factor VIII is also low; platelet count is normal.
  • Desmopressin (DDAVP) is the first-line treatment for many types because it releases stored vWF and factor VIII.
  • vWF/factor VIII concentrates are used for severe disease or when DDAVP is ineffective; aminocaproic acid helps mucosal bleeding.
  • Inheritance is most often autosomal dominant, so it affects males and females roughly equally.

Signs & symptoms

  • Mucosal bleeds: epistaxis, gums
  • Menorrhagia, easy bruising
  • Prolonged bleed after surgery

Nursing priorities

  1. Control mucosal bleeding with direct pressure; for epistaxis have the client sit up and lean forward and apply pressure.
  2. Administer DDAVP or vWF-containing factor concentrate as prescribed before procedures or for active bleeding.
  3. Implement bleeding precautions and avoid IM injections, aspirin, and NSAIDs.
  4. Monitor for and quantify blood loss, especially menstrual bleeding, and assess for anemia.
  5. Hold pressure on venipuncture and injection sites longer than usual.

Labs & values

Bleeding time / PFA closure time: prolonged
vWF antigen and activity (ristocetin cofactor): decreased
Factor VIII level: normal or decreased
Platelet count: normal
aPTT: normal or prolonged

Patient teaching

  • Avoid aspirin, NSAIDs, and other platelet-inhibiting medications; use acetaminophen for pain.
  • Women with heavy periods may benefit from hormonal therapy; report excessive menstrual bleeding.
  • Carry medical alert identification and notify dentists/surgeons before any procedure.
  • Apply prolonged pressure to cuts and nosebleeds; keep DDAVP available if prescribed.
  • Genetic counseling is appropriate as the condition is inherited.

Red flags: report now

  • Heavy or uncontrolled bleeding, signs of significant blood loss (tachycardia, hypotension, dizziness) require prompt reporting.
  • Never administer aspirin, NSAIDs, or antiplatelet agents.
  • Sudden severe internal bleeding signs (abdominal pain, GI bleeding) warrant immediate evaluation.

References

Clinical references for this topic

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