Von Willebrand disease vs Hemophilia
How to tell Von Willebrand disease and Hemophilia apart when a board question makes them look alike.
vWD is platelet-adhesion mucosal bleeding with abnormal ristocetin; hemophilia is coagulation-factor deep bleeding with hemarthroses.
Side by side
Von Willebrand disease causes mucocutaneous bleeding, epistaxis, menorrhagia, prolonged bleeding time, abnormal ristocetin test, and sometimes prolonged PTT from reduced factor VIII stabilization.
Hemophilia A or B causes deep tissue bleeding, hemarthroses, prolonged PTT, normal platelet count and bleeding time, and factor VIII or IX deficiency.
Deficient or dysfunctional vWF impairs platelet adhesion to subendothelium and decreases factor VIII stability.
Intrinsic pathway factor deficiency impairs fibrin clot formation, producing deep bleeding after trauma or spontaneously in severe disease.
Low vWF antigen/activity, abnormal ristocetin cofactor activity, prolonged bleeding time, normal platelet count, and possible prolonged PTT support vWD.
Low factor VIII in hemophilia A or low factor IX in hemophilia B with prolonged PTT and normal PT/bleeding time supports hemophilia.
The trap
Both can prolong PTT through factor VIII biology, so bleeding pattern and ristocetin/vWF testing separate them.
The repair move
For bleeding disorders, decide mucosal platelet-type bleeding versus deep coagulation-factor bleeding.
Try a free question
CoreA patient has recurrent epistaxis, heavy menstrual bleeding, easy bruising, prolonged bleeding time or abnormal platelet function analysis, abnormal ristocetin cofactor activity, normal platelet count, and mildly prolonged PTT. Which diagnosis is most likely?
Show answer and explanation
Answer: A. Von Willebrand disease. Mucocutaneous bleeding with abnormal ristocetin testing supports von Willebrand disease.
Why the other choices are tempting
- B. Hemophilia A causes factor VIII deficiency with deep bleeding and normal bleeding time.
- C. Hemophilia B causes factor IX deficiency with deep bleeding and normal bleeding time.
- D. ITP causes thrombocytopenia with petechiae.
- E. DIC causes prolonged PT/PTT, low fibrinogen, and thrombocytopenia.
Flashcards
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What bleeding pattern supports vWD?
Mucocutaneous bleeding such as epistaxis and menorrhagia supports vWD.
What bleeding pattern supports hemophilia?
Deep tissue bleeding and hemarthroses support hemophilia.
Quick answers
What is the fastest way to tell Von Willebrand disease from Hemophilia?
vWD is platelet-adhesion mucosal bleeding with abnormal ristocetin; hemophilia is coagulation-factor deep bleeding with hemarthroses.
What is the key clue for Von Willebrand disease?
Von Willebrand disease causes mucocutaneous bleeding, epistaxis, menorrhagia, prolonged bleeding time, abnormal ristocetin test, and sometimes prolonged PTT from reduced factor VIII stabilization.
What is the key clue for Hemophilia?
Hemophilia A or B causes deep tissue bleeding, hemarthroses, prolonged PTT, normal platelet count and bleeding time, and factor VIII or IX deficiency.
What is the common trap with Von Willebrand disease vs Hemophilia?
Both can prolong PTT through factor VIII biology, so bleeding pattern and ristocetin/vWF testing separate them.