Hemophilia vs Von Willebrand disease
How to tell Hemophilia and Von Willebrand disease apart when a board question makes them look alike.
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Deep joint or muscle bleeding points to hemophilia, while mucosal bleeding with low ristocetin activity points to von Willebrand disease.
Side by side
Deep tissue bleeding such as hemarthroses and muscle hematomas with prolonged PTT suggests hemophilia.
Mucocutaneous bleeding such as epistaxis or menorrhagia with prolonged bleeding time pattern suggests von Willebrand disease.
Hemophilia A or B is inherited deficiency of factor VIII or IX causing impaired intrinsic coagulation.
Von Willebrand disease is quantitative or qualitative vWF defect causing impaired platelet adhesion and relative factor VIII instability.
PT is normal and PTT is prolonged; factor VIII or IX activity is reduced, often below 40% in mild disease and much lower in severe disease, while vWF antigen is usually normal.
vWF antigen and ristocetin cofactor activity are reduced, PTT may be normal or mildly prolonged, and platelet count is typically normal.
The trap
A mildly prolonged PTT can lead to overcalling hemophilia in vWD, especially when menorrhagia is the main complaint. Bleeding pattern plus vWF testing is the practical discriminator before factor replacement decisions.
The repair move
Next best step: treat active hemophilia bleeding with factor replacement, for example recombinant factor VIII 25 to 50 IU/kg IV targeting hemostatic levels, and avoid intramuscular injections. For most type 1 vWD bleeding or procedures, use desmopressin 0.3 mcg/kg IV over 20 to 30 minutes if responsive, with vWF concentrate for severe or nonresponsive cases.
Try a free question
CoreA 19-year-old man has recurrent spontaneous hemarthroses of the ankles and knees. BP 120/70 mm Hg, HR 88/min, temp 36.8 C. PT is normal, PTT is prolonged, and factor VIII activity is 6%. What is the most likely diagnosis?
Show answer and explanation
Answer: B. Hemophilia A. Deep joint bleeding with markedly low factor VIII and prolonged PTT indicates hemophilia A.
Why the other choices are tempting
- A. vWD more often causes mucosal bleeding and low vWF studies rather than recurrent hemarthroses with very low factor VIII.
- C. ITP causes thrombocytopenic mucosal bleeding, not isolated intrinsic-factor deficiency.
- D. Factor V Leiden predisposes to thrombosis rather than bleeding.
- E. DIC would typically produce prolonged PT and thrombocytopenia with systemic illness.
Flashcards
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Which bleeding phenotype favors hemophilia over vWD?
Hemarthroses and deep muscle bleeding favor hemophilia.
Which lab test is central for diagnosing vWD?
vWF antigen and ristocetin cofactor activity are central diagnostic tests for vWD.
Quick answers
What is the fastest way to tell Hemophilia from Von Willebrand disease?
Deep joint or muscle bleeding points to hemophilia, while mucosal bleeding with low ristocetin activity points to von Willebrand disease.
What is the key clue for Hemophilia?
Deep tissue bleeding such as hemarthroses and muscle hematomas with prolonged PTT suggests hemophilia.
What is the key clue for Von Willebrand disease?
Mucocutaneous bleeding such as epistaxis or menorrhagia with prolonged bleeding time pattern suggests von Willebrand disease.
What is the common trap with Hemophilia vs Von Willebrand disease?
A mildly prolonged PTT can lead to overcalling hemophilia in vWD, especially when menorrhagia is the main complaint. Bleeding pattern plus vWF testing is the practical discriminator before factor replacement decisions.