Locations Main Campus: Chesterfield, MO 63017   |   Locations
314-434-1500 314-434-1500   |   Contact Us

Multimedia Encyclopedia


 
E-mail Form
Email Results

 
 
Print-Friendly
Bookmarks
bookmarks-menu

Von Willebrand disease

Bleeding disorder - von Willebrand

 

Von Willebrand disease is the most common hereditary bleeding disorder.

Causes

 

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.

A family history of a bleeding disorder is the primary risk factor.

 

Symptoms

 

Symptoms may include:

  • Abnormal menstrual bleeding
  • Bleeding of the gums
  • Bruising
  • Nosebleeds
  • Skin rash

Note: Most women with heavy or prolonged menstrual bleeding do not have von Willebrand disease.

 

Exams and Tests

 

Von Willebrand disease may be hard to diagnose. Low von Willebrand factor levels and bleeding do not always mean you have von Willebrand disease.

Tests that may be done to diagnose this disease include:

  • Bleeding time
  • Blood typing
  • Factor VIII level
  • Platelet function analysis
  • Platelet count
  • Ristocetin cofactor test
  • Von Willebrand factor specific tests

 

Treatment

 

Treatment may include DDAVP (desamino-8-arginine vasopressin), a medicine to raise von Willebrand factor level and reduce the chances for bleeding.

However, DDAVP does not work for all types of von Willebrand disease. Tests should be done to determine what type of von Willebrand you have. If you are going to have surgery, your doctor may give you DDAVP before surgery to see if your von Willebrand factor levels increase.

The drug Alphanate (antihemophilic factor) is approved to decrease bleeding in people with the disease who must have surgery or any other invasive procedure.

Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.

 

Outlook (Prognosis)

 

Bleeding may decrease during pregnancy. Women who have this condition usually do not have excessive bleeding during childbirth.

This disease is passed down through families. Genetic counseling may help prospective parents understand the risk to their children.

 

Possible Complications

 

Bleeding may occur after surgery or when you have a tooth pulled.

Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can make this condition worse. DO NOT take these medicines without first talking to your health care provider.

 

When to Contact a Medical Professional

 

Call your 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 providers about your condition.

 

 

References

Flood VH, Scott JP. Von Willebrand disease. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 477.

James P, Rydz N. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2013:chap 140.

Nichols WL. Von Willebrand Disease and hemorrhagic abnormalities of platelet and vascular function. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 173.

 
  • Blood clot formation

    Blood clot formation - illustration

    Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops. Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days.

    Blood clot formation

    illustration

  • Blood clots

    Blood clots - illustration

    Blood clots (fibrin clots) are the clumps that result when blood coagulates.

    Blood clots

    illustration

    • Blood clot formation

      Blood clot formation - illustration

      Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops. Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days.

      Blood clot formation

      illustration

    • Blood clots

      Blood clots - illustration

      Blood clots (fibrin clots) are the clumps that result when blood coagulates.

      Blood clots

      illustration

    A Closer Look

     

      Talking to your MD

       

        Self Care

         

          Tests for Von Willebrand disease

           

             

            Review Date: 2/1/2016

            Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

            The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
            adam.com

             
             
             

             

             

            A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.



            Content is best viewed in IE9 or above, Firefox and Google Chrome browser.