What are the symptoms of von Willebrand factor?

September 27, 2025 •

3 min read

Von Willebrand disease (VWD), caused by a deficiency or defect in von Willebrand factor (VWF), affects up to 1% of the U.S. population, making it the most common inherited bleeding disorder. The symptoms of von Willebrand factor deficiency vary widely in severity, from mild and unnoticeable to severe and life-threatening.

Quick Summary

Symptoms of von Willebrand disease range from easy bruising and frequent nosebleeds to more severe issues like heavy menstrual bleeding and prolonged bleeding after injury, surgery, or dental work, depending on the type and severity of the condition.

Key Points

Easy Bruising: Many people with VWD experience frequent, large, and sometimes lumpy bruises from minimal or no trauma.
Prolonged Nosebleeds: Frequent and long-lasting nosebleeds are a common symptom across different types of von Willebrand disease.
Heavy Menstrual Bleeding: Women with VWD often suffer from heavy or prolonged menstrual periods, which can lead to anemia.
Excessive Bleeding after Procedures: Prolonged bleeding after dental work, surgery, or childbirth is a key indicator of VWD.
Severity Varies by Type: Symptoms range from mild in Type 1 to severe, potentially including spontaneous joint and muscle bleeding, in the rare Type 3.
Management is Possible: While VWD is a lifelong condition, it can be effectively managed with medication and lifestyle adjustments.

Understanding Von Willebrand Disease (VWD)

Von Willebrand disease is a hereditary bleeding disorder that prevents blood from clotting correctly due to issues with the von Willebrand factor (VWF) protein. VWF is crucial for platelet function and for protecting factor VIII, another clotting protein. A deficiency or defect in VWF impairs the blood clotting process.

The Role of Von Willebrand Factor (VWF)

VWF, a glycoprotein produced in endothelial cells and megakaryocytes, facilitates platelet adhesion to injured blood vessels and to each other. It also carries and protects factor VIII, which is essential for the clotting cascade.

Common Symptoms Across All VWD Types

Excessive bleeding is the hallmark of VWD, presenting differently based on individual factors and severity. Common symptoms include:

Easy bruising, often resulting in large, lumpy bruises.
Frequent or prolonged nosebleeds lasting over 10 minutes.
Gum bleeding, especially during dental care.
Prolonged bleeding from minor cuts.
Heavy or prolonged menstrual bleeding (menorrhagia) in women, potentially causing anemia. Signs can include soaking through protection hourly on heavy days, periods lasting over 7 days, or passing large blood clots.

Distinguishing Symptoms by VWD Type

VWD is categorized into three main types based on VWF abnormalities, which influence symptom presentation and severity.

Type 1 VWD

The most common and typically mildest form, resulting from low VWF levels. Bleeding is often mild and mucocutaneous, though significant bleeding can follow surgery or trauma.

Type 2 VWD

This type involves dysfunctional VWF despite normal production. Subtypes exist:

Type 2A: Characterized by a lack of large VWF multimers, leading to moderate to severe bleeding.
Type 2B: A rare type where VWF binds excessively to platelets, causing their premature removal and potentially low platelet counts (thrombocytopenia).
Type 2M: VWF doesn't function correctly, but multimer size is normal. Symptoms resemble Type 1 but are often more severe.
Type 2N: A defect in VWF's ability to bind factor VIII, resulting in symptoms similar to mild hemophilia A, including muscle and joint bleeding.

Type 3 VWD

The rarest and most severe form, caused by a near absence of VWF and severe factor VIII deficiency. This leads to more pronounced symptoms, including spontaneous uncontrolled bleeding, severe joint and muscle bleeding (hemarthroses), and life-threatening hemorrhages.

Acquired Von Willebrand Syndrome (AVWS)

Rarely, VWD can be acquired later in life due to other medical conditions. Symptoms are similar to inherited VWD but typically appear in adulthood.

Comparing VWD to Hemophilia

Both VWD and hemophilia are bleeding disorders involving clotting factors, but they differ in key ways:


Feature	Von Willebrand Disease (VWD)	Hemophilia
Cause	Deficiency or defect of von Willebrand factor (VWF).	Deficiency of a specific clotting factor (VIII for Hemophilia A, IX for Hemophilia B).
Inheritance Pattern	Primarily autosomal dominant, Type 3 and some Type 2 are autosomal recessive.	X-linked recessive, primarily affecting males.
Primary Bleeding	Mucocutaneous bleeding (nose, gums, heavy menstrual periods, easy bruising) is most common.	Joint and muscle bleeding (hemarthrosis) are classic signs, especially in severe forms.
Diagnosis	Requires specialized blood tests, including VWF antigen, VWF activity, and Factor VIII levels.	Typically diagnosed with standard coagulation tests and specific factor level assays.
Prevalence	Most common inherited bleeding disorder.	Less common than VWD.

When to See a Doctor

Seek medical advice if you experience frequent, prolonged, or hard-to-stop nosebleeds, unusually heavy menstrual periods, easy bruising with large or lumpy bruises, or prolonged bleeding after minor procedures.

Diagnosis and Management

Diagnosis involves a medical history, physical exam, and specific blood tests to measure VWF levels and activity, and factor VIII levels. Multiple tests may be needed due to fluctuating VWF levels. Management is individualized and may include medications like desmopressin or replacement therapies, clot-stabilizing medicines, and hormonal therapies for heavy menstrual bleeding. Avoiding certain pain relievers is also important.

Conclusion

Recognizing the symptoms of von Willebrand factor deficiency is vital for timely diagnosis and management. Symptom severity varies widely, and even mild cases pose bleeding risks during procedures. Working with healthcare professionals, particularly a hematologist, can help manage VWD and enable individuals to lead active lives.

For more detailed information on living with von Willebrand disease, you can visit the CDC's resources.

Frequently Asked Questions

Von Willebrand factor (VWF) is a protein that serves two main functions in blood clotting: it helps platelets stick together and adhere to the blood vessel wall at the site of injury, and it carries and protects another crucial clotting protein, factor VIII.

Von Willebrand disease (VWD) is almost always inherited, though the pattern varies by type. Type 1 and most Type 2 are passed down in an autosomal dominant pattern (one affected gene copy is enough). The rare Type 3 is inherited in an autosomal recessive pattern (both copies must be affected).

The severity of symptoms depends on the type of VWD and the specific genetic mutation. Some types cause a mild quantitative deficiency of VWF (Type 1), while others cause a severe qualitative defect or a near-complete absence of the factor (Types 2 and 3), leading to more pronounced bleeding.

Yes, especially in its more severe forms. Type 3 VWD, due to its low factor VIII levels, can mimic severe hemophilia A, causing joint and muscle bleeding. However, the inheritance patterns and other clinical signs typically differ.

If you or a family member have a history of easy bruising, prolonged bleeding, or other potential symptoms, you should consult a doctor, preferably a hematologist, for evaluation and diagnosis. A detailed history and specific blood tests are necessary.

Management for heavy menstrual bleeding (menorrhagia) may include hormonal therapies like birth control pills or intrauterine devices, as well as antifibrinolytic medicines such as tranexamic acid, which helps stabilize clots.

Inherited VWD is a genetic condition present at birth, passed down through genes. Acquired VWD is a rare form that develops later in life, often associated with other medical conditions like certain cancers or autoimmune diseases.