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Von Willebrand's Disease


  • Von Willebrand's disease is a problem involving the body's ability to form blood clots.  Normally, blood clots form in response to bleeding-and blood clots functionally by stopping the flow of blood.  People with this disease bleed more easily.  Von Willebrand's disease is passed on from parent to child, affecting male and female alike.  Usually, the bleeding problems are mild.  In severe cases, treatment is available to facilitate normal blood clotting.

  • Nosebleeds, bleeding from their gums, or excessive bleeding during menstruation.  Some may have bleeding from the intestine.
  • Many have increased bleeding after surgery or after dental work (particularly tooth removal).
  • Severe bleeding and spontaneous bleeding into joints usually does not occur.
  • Aspirin use should be avoided, as it worsens bleeding problems.
  • Patients who are pregnant or are taking estrogen will have fewer bleeding problems

  • It is due to problems with a specific protein in the blood that helps the body form blood clots.  The protein is called vWf -- von Willebrand factor.
  • There are several subtypes of von Willebrand's disease, depending on whether there is too little vWf protein, or whether it does not work properly.

  • Usually, the blood count is normal.
  • The best test is measuring the bleeding time, checking for prolonged bleeding time
  • There are tests that directly measure the amount of vWf and also check to see how well it works.

  • Bleeding problems are usually mild and no treatment may be needed.
  • Aspirin (and other similar drugs) use should be avoided.
  • Treatment is needed prior to dental or surgical procedures so that bleeding is not excessive.
  • Replacement of the von Willebrand factor with fresh frozen plasma or cryoprecipitate
  • Those with Type I disease can be treated with a medicine called Desmopressin Acetate.  This medicine sometimes does not fully correct the Type I defect  and  should not be used in patients with Type IIa or IIb disease.
  • Those who cannot be treated with Desmopressin may be given vWF intravenously to effect normal clotting.

  • Glanzmann's thrombasthenia
  • Bernard-Soulier syndrome
  • Storage pool disease
  • Hemophilia
  • Blood cancers
  • Kidney failure
  • Aspirin or other anti-inflammatory use

  • Prognosis is excellent.  Most patients can be managed easily, once diagnosed.

  • Special Information
    1. Because this disease is inherited, all family members of patients with this disease should see their doctor and be evaluated for this condition.
    2. Normally, screening for this disease is done early, with favorable results.















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