Acquired Von Willebrand Syndrome
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GENERAL INFORMATION:
What is acquired von Willebrand syndrome?
- Acquired von Willebrand syndrome (AVWS) is also called acquired von Willebrand disease. AVWS is a rare bleeding disorder that is not inherited from your parents and usually develops later in life. Normally, blood cells and different substances help the body form clots and stop you from bleeding too much. These cells and substances include platelets, calcium, vitamin K, fibrinogen (protein), and von Willebrand factor (VWF). The VWF is a glue-like protein produced by the cells that line the blood vessel walls. Blood also carries another clotting protein called factor VIII. All of these substances stick to the cut in the blood vessel and create a plug. This plug prevents further bleeding and stays there until the damaged blood vessel heals.
- With AVWS, there is a problem with the von Willebrand factor. Although the VWF is produced normally, it may not work correctly, or it may be present only in small amounts. The platelets will not be able to plug the cut in the blood vessel without the VWF. The blood will take a longer time to clot and wound repair will be delayed. When this happens, the bleeding from the injured vessel may continue outside or inside the body. Internal bleeding may form bruises and decrease blood flow, leading to a lack of oxygen to body organs.
What causes acquired von Willebrand syndrome? The following are possible causes and conditions that may increase your risk of having AVWS:
- Diseases: The VWF may stick or attach itself to cancer (tumors), such as in leukemia, lymphomas, or kidney tumors. This will decrease the amount of VWF going around in the body. Certain diseases, such as scleroderma, lupus, or heart diseases, may also increase your risk. VWF is decreased or absent in hypothyroidism. Hypothyroidism is a condition where the thyroid gland produce little thyroid hormone (chemical) which helps release VWF.
- Immune problems: The immune system is the part of your body that fights infection. A problem with the immune system sometimes makes your body attack its own cells. This may make you more likely to develop AVWS.
- Medicines: Medicines used to treat cancer, depression, or infections may cause AVWS. Ask your caregiver for information about these medicines.
- Toxic chemicals: Frequent exposure to toxic chemicals, such as pesticides, may increase your risk of having AVWS.
What are the signs and symptoms of acquired von Willebrand syndrome? Signs and symptoms of AVWS are similar to congenital (born with) VWD. Easy bruising and bleeding in the muscles, joints, or soft tissue areas are not that common in AVWS. You may also have any of the following:
- Bleeding too much or too long from wounds or incisions (cuts) after having surgery or teeth pulled.
- Frequent bleeding from the gums or nose.
- Stools (bowel movements) that are black or dark.
- Urine that is pinkish or reddish in color.
- Women may have heavy menstrual periods, or too much bleeding when giving birth.
How is acquired von Willebrand syndrome diagnosed? Your caregiver will take your past and present health history. He may want to know the details of your bleeding episodes. Your caregiver may also need to know if other family members have von Willebrand disease or other bleeding problems. Your blood will also be taken for different tests. These will check the amount and structure of the VWF in your blood. Your caregiver may also need to know how well your VWF, platelets, and other clotting factors work. Blood tests may need to be repeated often since VWF or factor VIII may change over time.
How is acquired von Willebrand syndrome treated? Treatment of AVWS includes treating its underlying cause. This may include treating a cancer or stopping a certain medicine for a while. The aim of therapy is to control and prevent the bleeding problem. You may also need one or more of the following:
- Medicine:
- Immune globulins: Immune globulins can be used to treat many different problems. It may be given to help your immune system fight infection. It may also help if your body does not produce enough of certain kinds of blood cells. This medicine may help if your system fights something in your blood or body that it should not. Ask your caregiver for more information about how immune globulin medicine may help you.
- Desmopressin: This medicine, also called DDAVP, boosts the amount of VWF and factor VIII in your blood. Ask your caregiver for more information about desmopressin if you need to use this medicine.
- Steroids: Steroid medicine may be given to decrease inflammation, which is redness, pain, and swelling.
- Immune globulins: Immune globulins can be used to treat many different problems. It may be given to help your immune system fight infection. It may also help if your body does not produce enough of certain kinds of blood cells. This medicine may help if your system fights something in your blood or body that it should not. Ask your caregiver for more information about how immune globulin medicine may help you.
- Replacement therapy: A factor VIII concentrate that is rich in VWF may be needed to control or prevent bleeding. This is given by injection or infusion (fluid given through a vein). Factor concentrates help the blood clot and stop bleeding following accidents, surgery, or other bleeding. In severe AVWS, this therapy may also be given regularly every few days to prevent bleeding. If you refuse a blood replacement, your condition may get worse. Ask your caregiver for more information on receiving blood concentrates.
Where can I find support and more information? Having AVWS may be life-changing for you and your family. Accepting that you have AVWS may be hard. You and those close to you may feel sad, angry or frightened. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a bleeding disorder support group. This is a group of people who have bleeding problems. Ask your caregiver for the names and numbers of support groups. Contact the following for more information:
- American Academy of Family Physicians
PO Box 11210
Shawnee Mission, KS 66207-1210
Phone: 1-913-906-6000
Web Address: http://www.aafp.org
- National Center on Sleep Disorders Research
National Heart, Lung and Blood Institute, NIH
6705 Rockledge Drive
Bethesda, MD 20892-7993
Phone: 1-301-435-0199
Phone: 1-800-575-9355
Web Address: http://www.nhlbi.nih.gov/about/ncsdr/
CARE AGREEMENT:
You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.
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