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Von Willebrand Disease — RACP Paediatrics MCQ

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HardHaematology & OncologyVon Willebrand DiseaseRACP Paediatrics

A 7-year-old boy presents with recurrent nosebleeds and easy bruising. His platelet count and PT are normal. APTT is prolonged at 52 seconds (normal 25–35). Factor VIII level is 12% (normal 50–150%). What is the most likely diagnosis?

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Correct answer: BVon Willebrand disease type 1

Von Willebrand disease (VWD) type 1 is the most common inherited bleeding disorder. It presents with mucocutaneous bleeding (epistaxis, bruising, menorrhagia). VWF is a carrier protein for factor VIII, so VWD often causes a mildly prolonged APTT and mildly reduced FVIII levels. Confirmatory testing includes VWF antigen, VWF activity (ristocetin cofactor), and FVIII levels.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Bleeding Disorders