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General Medicine · Haematology: von Willebrand disease
Sample itemA 20-year-old woman has heavy menstrual bleeding and recurrent nosebleeds since childhood. Platelet count and PT are normal, the aPTT is mildly prolonged, and the bleeding time is prolonged. Factor VIII activity is 30%, von Willebrand factor antigen is 28% and ristocetin cofactor activity is low in proportion. What is the most appropriate treatment for a heavy menstrual bleed?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Desmopressin with tranexamic acid
Type 1 von Willebrand disease is a quantitative partial deficiency of von Willebrand factor, which carries factor VIII and mediates platelet adhesion. Desmopressin releases stored von Willebrand factor from endothelial Weibel-Palade bodies and raises factor VIII, and tranexamic acid stabilises clots in mucosal bleeding. Von Willebrand factor concentrates are used for severe bleeding or non-responders.
Option by option
A. Desmopressin with tranexamic acid (correct)
Correct. Desmopressin releases endogenous von Willebrand factor and tranexamic acid reduces mucosal bleeding.
B. Platelet transfusion
Platelet counts are normal and the defect is in von Willebrand factor, so platelet transfusion is not needed.
C. Vitamin K
Vitamin K corrects deficiency of factors II, VII, IX and X, which is not present here.
D. Fresh frozen plasma
Plasma carries a risk of volume overload and is not the preferred source of von Willebrand factor when concentrates are available.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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