Von Willebrand disease (patient information)
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Editor-in-Chief: C. Michael Gibson, M.S.,M.D. [1] Phone:617-632-7753; Associate Editor-In-Chief: Lakshmi Gopalakrishnan, M.B.B.S.
Overview
Von Willebrand disease is the most common inherited bleeding disorder. It is usually associated with mild bleeding.
What are the symptoms of Von Willebrand disease?
- Abnormally heavy menstrual bleeding
- Bleeding of the gums without apparent cause
- Easy bruising
- Nosebleeds
- Skin rash
- Prolonged bleeding after dental work
What causes Von Willebrand disease?
- Von Willebrand disease is caused by a deficiency of von Willebrand factor. Von Willebrand factor helps blood platelets clump together and stick to the blood vessel wall, which is necessary for normal blood clotting. There are several types of Von Willebrand disease.
- Von Willebrand disease affects men and women equally. Most cases are mild. Bleeding may occur after surgery or when you have a tooth pulled. Aspirin and other nonsteroidal anti-inflammatory drugs like ibuprofen or naproxen can make this condition worse.
Who is at highest risk?
- A family history of a bleeding disorder is the primary risk factor.
- In women with heavy or prolonged menstrual bleeding, von Willebrand is more common in Caucasian women than in African American women. The majority of women with heavy or prolonged menstrual bleeding do NOT have Von Willebrand disease.
When to seek urgent medical care?
- Call your health care provider if bleeding occurs without reason.
- If you have von Willebrand disease and are scheduled for surgery or are in an accident, be sure you or your family notify the health care providers about your condition.
Diagnosis
- Tests that may be done to diagnose this disease include:
- Bleeding time (is prolonged)
- Platelet aggregation test
- Platelet count (may be low or normal)
- Ristocetin cofactor test (the primary assay test used to diagnose von Willebrand disease)
- Von Willebrand factor level (level is reduced)
- This disease may also alter the results of the following tests:
- Factor VIII level
- Von Willebrand factor multimers (parts of the von Willebrand factor protein molecule)
- Von Willebrand disease may be hard to diagnose. Low von Willebrand factor levels and bleeding do not always mean you have von Willebrand disease.
Treatment options
- Most people do not require any treatment
- Medications such as desamino-8-arginine vasopressin (DDAVP) can be given to raise the levels of von Willebrand factor, which will reduce the tendency toward bleeding.
- Some types of von Willebrand disease do not respond to DDAVP. Tests should be done to determine a patient's specific type of von Willebrand disease before trauma or surgery occurs. A trial of DDAVP can be done before surgery to test whether von Willebrand factor levels increase.
- The drug Alphanate (factor VIII) is approved to decrease bleeding in patients with the disease who must have surgery or any other invasive procedure.
Where to find medical care for Von Willebrand disease?
Directions to Hospitals Treating Von Willebrand disease
What to expect (Outlook/Prognosis)?
- Prognosis is overall very good, most patients have mild forms of the disease and can expect normal lifespan.
- Patients with more severe forms of the disease may require treatment and need to avoid contact sports, but prognosis is still generally good.
- Women who have this condition generally do NOT have excessive bleeding during childbirth.
- This disease is passed down through families. Therefore, genetic counseling may help prospective parents understand the risk to their children.
Possible complications
- Bleeding (hemorrhaging) may occur after surgeries or other invasive procedures.
- If you have von Willebrand disease, do not take nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen, without talking to your health care provider as these can increase bleeding risk. Acetaminophen (tylenol) does not cause increased bleeding risk.