What is von Willebrand disease?
Von Willebrand disease is an inherited bleeding disorder. In order for blood to clot normally, several different proteins are needed in the blood. One of the most important of these proteins is von Willebrand factor.
In people with this disease, there is low levels of von Willebrand factor in the blood, or the factor does not work properly. Von Willebrand factor helps factor VIII and platelets to “stick together” at the site of injury and form a blood clot. If this factor is low or not working properly, the clot does not form properly and bleeding will last longer.
The disease is named after the Finnish physician Erik von Willebrand, who first described it.
Von Willebrand disease is the most common inherited bleeding disorder, estimated to affect about 1 percent of the population. It occurs equally in men and women, but women often have more pronounced symptoms due to menstruation and menstrual bleeding.
Symptoms of von Willebrand disease
Symptoms can range from mild to severe and vary greatly from person to person. Important signs include:
• Frequent nosebleeds (more than 5 times a year) lasting more than 10 minutes
• Prolonged bleeding from small cuts and wounds (more than 10 minutes)
• Easy bruising, especially large, prominent bruises
• Being told you are “anemic” or have been treated for anemia many times
• Heavy bleeding after any surgery or dental procedures
• Having one of these symptoms in several family members
• Having a relative diagnosed with a bleeding disorder, such as von Willebrand’s disease or hemophilia
Specific symptoms in women and girls
In women and girls, important symptoms include:
• Heavy, prolonged menstrual periods (needing to change a pad or tampon every hour, or bleeding lasting more than 7 days)
• Heavy bleeding after childbirth or after a miscarriage
If you or a member of your family has these symptoms, it is necessary to seek professional examination and advice.
Types of von Willebrand disease
Classically, the disease is divided into three main types, and there is also an “acquired” type:
- Type 1
• The most common type of the disease; about 60 to 80 percent of cases
• In this type, the amount of von Willebrand factor in the blood is reduced, but not completely eliminated.
• The severity of symptoms is usually mild. - Type 2
• Makes up about 15 to 30 percent of patients.
• Factor levels may be normal or near normal in the blood, but von Willebrand factor is not working properly.
• Type 2 itself has several subtypes, each showing a specific pattern of factor dysfunction.
• Symptoms are usually mild to moderate. - Type III
• A rare but severe form of the disease (about 5 to 10 percent of cases).
• In this type, the level of von Willebrand factor in the blood is very low or almost zero.
• Factor VIII levels may also be reduced, and bleeding may occur spontaneously, severely, and into joints and muscles. - Acquired type
• In this type, a person is not born with von Willebrand disease; rather, they develop a deficiency or dysfunction of von Willebrand factor later due to other diseases (such as some autoimmune, heart, or cancer diseases) or the use of certain medications.
Diagnosis of von Willebrand disease
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In some people, especially women, von Willebrand factor levels may rise and fall under different conditions (stress, physical activity, pregnancy, taking birth control pills, etc.); for this reason, tests may need to be repeated at different times.
The best place to diagnose and follow up on this disease is specialized centers for bleeding disorders and comprehensive hemophilia treatment centers, which provide the opportunity for specialized tests and consultation with experienced teams.
Treatment of von Willebrand disease
The type of treatment depends on the type of disease and the severity of symptoms. The main methods are:
• Von Willebrand factor release-stimulating drugs (such as desmopressin) in some mild and moderate types, especially in the type of single injection of concentrate containing von Willebrand factor and factor VIII in severe cases, in surgeries, serious bleeding or in types that do not respond to desmopressin
• Use of anti-clot dissolving drugs (antifibrinolytics) to reduce bleeding, especially in oral and menstrual bleeding
• In women, use of hormonal treatments (such as some pills and IUDs containing hormones) to control heavy menstruation
• Careful planning of surgical and dental procedures in coordination with a specialized center
Some patients with mild cases may not require treatment most of the time and may only receive treatment in special circumstances (surgery, severe trauma, childbirth, etc.).
Living with von Willebrand disease
Many affected individuals, with proper awareness and care, can lead active and normal lives. Important points:
• Familiarize the patient and family with the danger signs and when to see a doctor
• Inform the dentist and other doctors of the diagnosis of the disease before any treatment
• In women and girls, pay close attention to heavy menstruation and plan for pregnancy and childbirth in collaboration with a hematologist and gynecologist
• Conduct genetic counseling in families with several affected individuals
• Regular follow-up at a specialized center for bleeding disorders to adjust the type and amount of treatment
If you or a family member is experiencing prolonged bleeding, frequent bruising, frequent nosebleeds, or very heavy periods, consulting a specialist center can help with early diagnosis and prevention of complications.