Yes, you can get von Willebrand later in life: Understanding acquired von Willebrand syndrome (AVWS)

October 1, 2025 •

3 min read

While von Willebrand disease (VWD) is almost always inherited, and many with mild cases may not be diagnosed until adulthood, it is possible to develop a form of the disorder later in life as a result of other medical conditions, known as acquired von Willebrand syndrome (AVWS). Unlike its genetic counterpart, AVWS is not passed down through families.

Quick Summary

Acquired von Willebrand syndrome is a rare bleeding disorder that develops in adults, often due to underlying health problems affecting the immune system, blood, or heart.

Key Points

Acquired vs. Inherited: It is possible to develop a bleeding disorder that mimics von Willebrand disease (VWD) later in life, known as acquired von Willebrand syndrome (AVWS), which is not hereditary.
Underlying Causes: AVWS is almost always a consequence of another underlying medical condition, most commonly affecting the immune system, heart, or blood.
Diverse Mechanisms: The development of AVWS is complex and can be caused by autoantibodies attacking VWF, increased mechanical shear stress, or adsorption of VWF onto other cells.
Key Diagnostic Clue: Unlike inherited VWD, a patient with AVWS typically has no personal or family history of bleeding problems prior to the onset of symptoms.
Treatment Focus: The most crucial aspect of managing AVWS is identifying and treating the underlying medical condition, which can potentially cure the syndrome.
Tailored Therapy: Treatment for bleeding episodes in AVWS varies and may include IVIG, desmopressin, or factor replacement, depending on the specific cause and mechanism.

Inherited vs. Acquired: What's the Difference?

Von Willebrand disease (VWD) is a common inherited bleeding disorder caused by a genetic mutation affecting von Willebrand factor (VWF), a protein crucial for blood clotting. Acquired von Willebrand syndrome (AVWS), however, is a rare, non-hereditary condition that develops later in life due to an underlying medical issue, not genetics.

The Mechanisms Behind Acquired VWD

AVWS can result from several mechanisms that impair VWF function, often depending on the associated medical condition. These mechanisms include the immune system producing autoantibodies that clear or interfere with VWF (seen in autoimmune and lymphoproliferative disorders), increased mechanical stress destroying VWF in cardiovascular conditions, adsorption of VWF onto abnormal blood cells in certain cancers, and, rarely, decreased VWF production due to conditions like hypothyroidism.

Underlying Diseases Associated with AVWS

AVWS is linked to other health problems, particularly those affecting the blood, heart, or immune system. Common associated conditions include lymphoproliferative disorders (such as multiple myeloma or CLL), cardiovascular disorders (like aortic stenosis), myeloproliferative disorders (including essential thrombocythemia), autoimmune disorders (such as lupus), and other issues like hypothyroidism.

Symptoms and Diagnosis of AVWS

The primary symptom of AVWS is an increased tendency to bleed, similar to inherited VWD, with severity varying. Symptoms can include easy bruising, frequent nosebleeds, heavy menstrual bleeding, prolonged bleeding after procedures, and sometimes gastrointestinal bleeding. Diagnosis is challenging due to overlapping features with inherited VWD. A lack of prior bleeding issues or family history of VWD is a key indicator of AVWS. Diagnosis involves specialized blood tests to evaluate VWF levels and function.

Comparison of Inherited vs. Acquired VWD


Feature	Inherited von Willebrand Disease (VWD)	Acquired von Willebrand Syndrome (AVWS)
Onset	Present at birth, though often not diagnosed until later in life, especially in mild cases.	Develops later in life, typically in adulthood.
Cause	Genetic mutation affecting the VWF gene.	Associated with an underlying medical condition (e.g., autoimmune, cardiovascular, cancer).
Family History	Often a positive family history of bleeding disorders.	Negative personal and family history of bleeding disorders.
Pathogenesis	Defect in the synthesis or structure of the VWF protein.	Inactivation, clearance, or destruction of a normally-produced VWF protein.
Mechanisms	Quantitative or qualitative defect of VWF, depending on the type.	Autoantibodies, adsorption onto cells, increased shear stress, or decreased synthesis.
Treatment	Treatments focus on replacing VWF or promoting its release from storage sites.	Treating the underlying disease is the most important step; additional hemostatic therapy may be needed.
Heritability	Can be passed on to children.	Cannot be passed on to children.

Treatment of Acquired VWD

The primary treatment for AVWS is addressing the underlying condition, which can potentially resolve the syndrome. Additional treatments for bleeding include IVIG (for antibody-mediated cases), desmopressin (effectiveness varies), VWF-containing concentrates (for severe bleeding), and antifibrinolytic agents to stabilize clots.

Conclusion

While inherited VWD is a lifelong condition, you can get von Willebrand later in life through acquired von Willebrand syndrome (AVWS). This rare disorder arises from underlying health problems like autoimmune, cardiovascular, or hematologic diseases. The mechanisms differ significantly from inherited VWD, primarily involving the accelerated destruction or clearance of VWF rather than a genetic defect. Diagnosis requires careful evaluation of both clinical and laboratory findings, especially a negative family history. Critically, treating the underlying disease is the most effective way to resolve AVWS, although specific treatments for bleeding are also available. Awareness of AVWS is growing, leading to more accurate diagnoses and better management for those with new-onset bleeding symptoms. For more detailed information on bleeding disorders, visit the CDC website.

Frequently Asked Questions

AVWS is a rare bleeding disorder that develops in individuals with no prior history of bleeding problems. Unlike the more common inherited form, it is caused by another underlying medical condition, such as an autoimmune or cardiovascular disease, that interferes with the function of the von Willebrand factor (VWF).

AVWS can be caused by several conditions, including lymphoproliferative disorders (e.g., multiple myeloma, MGUS), myeloproliferative disorders, autoimmune diseases (e.g., systemic lupus erythematosus), and cardiovascular problems (e.g., aortic stenosis).

AVWS is not genetic and appears later in life, usually in adulthood. Inherited VWD is present from birth due to a genetic mutation and runs in families. A person with AVWS cannot pass the condition to their children.

Symptoms of AVWS are similar to inherited VWD and include easy bruising, frequent nosebleeds, prolonged bleeding after a cut or surgery, and heavy menstrual periods in women. In some severe cases, bleeding into the joints or muscles can occur.

Diagnosing AVWS typically begins with a patient and family history showing no prior bleeding issues. This is followed by specialized blood tests to measure the quantity and function of the von Willebrand factor (VWF), as well as to identify any underlying causative medical condition.

AVWS is potentially curable if the underlying medical condition causing it can be effectively treated or removed. For example, a successful treatment for an associated cancer or surgical repair of a heart valve defect may resolve the AVWS.

Treatment focuses on controlling bleeding and addressing the underlying cause. Options include medications like desmopressin (DDAVP), intravenous immunoglobulin (IVIG), and factor replacement therapy with VWF concentrates. Antifibrinolytic agents may also be used to help stabilize clots.