ConditionBlood & immune system
Von Willebrand Disease
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting up to 1 in 100 people, though many have mild symptoms and are never diagnosed. It is caused by low levels or poor function of von Willebrand factor, a blood protein that helps platelets stick to damaged blood vessels and carries clotting factor VIII. Typical signs are frequent or long nosebleeds, easy bruising, bleeding gums, heavy menstrual periods, and prolonged bleeding after dental work, surgery or childbirth. Desmopressin, factor replacement and tranexamic acid control bleeding.
Also called: VWD, von Willebrand's disease, inherited bleeding disorder, von Willebrand factor deficiency
At a glance
- What it is
- An inherited shortage or malfunction of von Willebrand factor (VWF).
- How common
- Up to 1% of people; symptomatic disease about 1 in 1,000.
- Types
- Type 1 (low VWF, most common), type 2 (VWF does not work properly), type 3 (almost no VWF, rare and severe).
- Symptoms
- Nosebleeds, bruising, gum bleeding, heavy periods, bleeding after procedures or childbirth.
- Treatment
- Desmopressin, VWF concentrates, tranexamic acid, hormonal treatment for heavy periods.
When to get help
Call 911 or go to the ER
- Bleeding that will not stop after 15 to 20 minutes of firm pressure, or heavy bleeding with dizziness or fainting
- A head injury, or severe headache, in someone with VWD, especially type 3
- Vomiting blood, black stools or heavy rectal bleeding
- Heavy bleeding after childbirth, soaking a pad every hour
See a doctor within a day or two
- Soaking a pad or tampon every hour for several hours, or periods with large clots and dizziness
- A swollen, painful joint or muscle after minor injury (possible internal bleeding)
- Bleeding after dental work or surgery that keeps restarting
Book a routine appointment
- Frequent nosebleeds lasting more than 10 minutes, easy bruising, or heavy periods since your first period
- A family history of a bleeding disorder before surgery, dental work or pregnancy
- Unexplained iron-deficiency anemia
How von Willebrand factor works
When a blood vessel is injured, von Willebrand factor acts like glue, linking platelets to the damaged vessel wall to form an initial plug. It also carries and protects clotting factor VIII in the blood. Low or faulty VWF therefore causes bleeding mainly from mucous membranes (nose, gums, uterus, gut) and, when factor VIII is also very low, bleeding into joints and muscles.
Types of von Willebrand disease
| Type | Problem | Severity | Inheritance |
|---|---|---|---|
| Type 1 | Reduced amount of normal VWF | Mild to moderate; about 75% of cases | Autosomal dominant |
| Type 2 (2A, 2B, 2M, 2N) | VWF does not work properly | Moderate | Usually autosomal dominant |
| Type 3 | VWF almost absent, low factor VIII | Severe, similar to hemophilia | Autosomal recessive |
| Acquired VWD | Develops later due to other conditions | Varies | Not inherited; linked to heart valve disease, some cancers, hypothyroidism |
Unlike hemophilia, VWD affects males and females equally, but women are more often diagnosed because of heavy periods and childbirth.
Symptoms
- frequent or prolonged nosebleeds, longer than 10 minutes
- easy bruising, with bruises larger than a coin from minor bumps
- bleeding gums after brushing or flossing
- prolonged bleeding from small cuts
- heavy menstrual bleeding: periods longer than 7 days, soaking pads every 1 to 2 hours, clots larger than a quarter, iron deficiency
- bleeding after dental extraction, tonsillectomy or surgery
- heavy bleeding after childbirth
- in type 3: joint and muscle bleeds
Many people with type 1 VWD only discover it after unexpected bleeding with surgery or childbirth.
Diagnosis
- bleeding history, often using a standardized bleeding assessment tool
- family history
- blood tests: - VWF antigen (amount of VWF) - VWF activity (platelet-binding function) - factor VIII activity - blood count and platelet count - multimer analysis and other tests to identify type 2 subtypes
- tests may need repeating, since VWF levels rise with stress, exercise, inflammation, pregnancy and estrogen
Teenagers and women with heavy periods since their first period should be screened for bleeding disorders.
Treatment
Desmopressin (DDAVP)
A synthetic hormone given by nasal spray or injection that releases stored VWF and factor VIII. It works well for most type 1 and some type 2 VWD. A test dose confirms response. Fluid intake must be limited for 24 hours afterward to avoid low sodium. It is not used in type 2B or type 3.
VWF concentrates
Plasma-derived or recombinant VWF given intravenously for type 3, many type 2 cases, major surgery, or when desmopressin does not work. Some people with severe disease use regular preventive infusions.
Antifibrinolytic medicines
Tranexamic acid and aminocaproic acid stabilize clots and are especially useful for nosebleeds, dental work and heavy periods.
Heavy menstrual bleeding
- hormonal birth control or the levonorgestrel IUD
- tranexamic acid during periods
- iron supplements for iron-deficiency anemia
Practical measures
- avoid aspirin and NSAIDs such as ibuprofen; use acetaminophen (paracetamol) for pain
- tell dentists and surgeons before any procedure, so a plan can be made
- wear a medical alert ID if you have moderate or severe VWD
- plan pregnancy and delivery with a hematologist and obstetrician
Outlook
Most people with VWD live normal lives with occasional treatment around procedures. Severe forms need specialist care at a hemophilia treatment center. Recognizing the condition and planning ahead prevents most serious bleeding.
Frequently asked questions
What are the symptoms of von Willebrand disease?
The most common symptoms are frequent or long nosebleeds, easy bruising, bleeding gums, heavy menstrual periods, and prolonged bleeding after cuts, dental work, surgery or childbirth. Severe forms can cause joint and muscle bleeding.
Is von Willebrand disease the same as hemophilia?
No. Both are inherited bleeding disorders, but VWD is caused by a problem with von Willebrand factor, affects men and women equally, and usually causes mucous membrane bleeding. Hemophilia is caused by a lack of factor VIII or IX and mainly affects males.
How is von Willebrand disease treated?
Treatment includes desmopressin, which releases the body's stored VWF, VWF concentrate infusions for more severe disease, and tranexamic acid to stabilize clots. Heavy periods are often treated with hormonal therapy. Many people only need treatment around surgery or bleeding episodes.
Can von Willebrand disease cause heavy periods?
Yes. Heavy menstrual bleeding is one of the most common symptoms in women with VWD, often starting with the first period. Women with very heavy periods, especially with other bleeding symptoms, should be tested for bleeding disorders.
Sources
This guide was written from current patient and clinical guidance, including:
- American Society of Hematology/International Society on Thrombosis and Haemostasis/National Hemophilia Foundation/World Federation of Hemophilia: Guidelines on the diagnosis and management of von Willebrand disease
- National Heart, Lung, and Blood Institute: Von Willebrand disease
- CDC: Von Willebrand disease
- American College of Obstetricians and Gynecologists: Screening and management of bleeding disorders in adolescents with heavy menstrual bleeding
- MedlinePlus: Von Willebrand disease