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Pathology
Low or abnormal vWF resulting in low levels of factor VIII and disorders of platelet function
Aetiology
Autosomal dominant inheritance
Signs
Intermittent mucocutaneous bleeding
Symptoms
Menorrhagia, Epistaxis
Investigations
Clotting screen: Prolonged APTT, normal PT, Low vWF levels and function.
Normal Factor VIII, Increased bleeding time
Treatment
Medical: Desmopressin (can increase vWF levels), vWF rich factor VIII
vWF concentrate, Tranexamic acid, fresh frozen plasma
Prognosis
Usually very good
Excessive bleeding
Key Facts
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Key Images
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Key References
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