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Von Willebrand Disease — RACP Adult Medicine MCQ

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ModerateHaematologyVon Willebrand DiseaseRACP Adult Medicine

A 25-year-old woman presents with menorrhagia, easy bruising, and prolonged bleeding after dental extraction. APTT is mildly prolonged. Factor VIII is mildly reduced (35%). Ristocetin cofactor activity is reduced. VWF antigen is low. Platelet count and function are normal. What is the most likely diagnosis?

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Correct answer: A — Von Willebrand disease

Mucocutaneous bleeding (menorrhagia, bruising, post-dental extraction bleeding) with mildly prolonged APTT, low VWF antigen, reduced ristocetin cofactor activity, and secondary reduction in factor VIII is von Willebrand disease. VWF carries and stabilises factor VIII. Type 1 (quantitative deficiency) is most common (75%). Treatment is DDAVP (desmopressin) for type 1, VWF concentrate for other types.

Reference: AHCDO – 2024 – VWD Guidelines; ASH ISTH 2021 VWD