Pathology refers to the low or abnormal levels of von Willebrand factor (vWF) which ultimately result in low levels of Factor VIII and disorders of platelet function.
Aetiology is associated with autosomal dominant inheritance.
Signs include intermittent mucocutaneous bleeding.
Clotting screen analyses typically reveal a prolonged APTT, a normal PT, low vWF levels and function, normal Factor VIII, and increased bleeding time.
Medical treatments for von Willebrand disease include Desmopressin (which may be used to increase vWF levels), vWF rich Factor VIII, vWF concentrate, Tranexamic acid, and Fresh Frozen Plasma.
Excessive bleeding is a possible complication of this condition.
Fortunately, the prognosis is usually very good.
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