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PubMed This is a summary of 24 peer-reviewed journal articles Updated
Hematology · Acquired Von Willebrand Syndrome

Can You Develop Von Willebrand Disease Later in Life?

At a Glance

Yes, you can develop a bleeding disorder later in life called Acquired Von Willebrand Syndrome (AVWS). Unlike genetic Von Willebrand Disease, AVWS is caused by other medical issues, such as heart valve problems, blood cancers, or autoimmune conditions that destroy the von Willebrand factor protein.

In this answer

3 sections

01

What Causes Acquired Von Willebrand Syndrome?

02

How is AVWS Different from Genetic VWD?

03

Safety and Daily Management While Waiting for Treatment

Yes, you can develop a condition that is nearly identical to Von Willebrand Disease later in life, even if you were not born with it. While standard Von Willebrand Disease (VWD) is an inherited genetic condition present from birth, the non-genetic form is known as Acquired Von Willebrand Syndrome (AVWS) [1].

Unlike genetic VWD, people who develop AVWS usually have no personal or family history of abnormal bleeding until they are older [2][3]. AVWS is a secondary condition, meaning it is triggered by another underlying medical issue that destroys, absorbs, or blocks the von Willebrand factor (VWF) protein in your blood [4][5].

What Causes Acquired Von Willebrand Syndrome?

AVWS happens when another disease or physical mechanism interferes with your body’s von Willebrand factor. The most common underlying causes fall into three main categories:

1. Heart and Blood Flow Issues

High physical stress on your blood (called shear stress) can stretch out the VWF protein, making it easily chopped up by enzymes in the bloodstream [6][7]. This mechanical destruction frequently happens in:

  • Severe aortic stenosis: A narrowing of the heart’s aortic valve. When this causes AVWS and gastrointestinal (stomach or intestine) bleeding, it is specifically known as Heyde’s syndrome [8][9].
  • Mechanical heart devices: Left ventricular assist devices (LVADs) or ECMO machines [10][11].

2. Blood Disorders and Cancers

Certain cancers and blood conditions can cause the body to clear VWF from the blood too quickly. This can happen because the cancer cells or extra blood cells physically absorb the VWF protein, or because the body creates antibodies that attack it [12][13]. Associated conditions include:

  • Monoclonal gammopathy of undetermined significance (MGUS) and Multiple Myeloma [5].
  • Lymphoproliferative disorders (such as certain lymphomas) [14][15].
  • Essential thrombocythemia: A condition where the body produces too many platelets, which can trap the VWF [16][17].

3. Autoimmune and Endocrine Conditions

Sometimes the immune system mistakenly targets the VWF protein with autoantibodies, neutralizing it or clearing it from the body [14][18]. Certain endocrine issues, particularly hypothyroidism (underactive thyroid), are also known causes of AVWS [19].

How is AVWS Different from Genetic VWD?

The symptoms of AVWS—such as frequent nosebleeds, unexplained gastrointestinal bleeding, easy bruising, or heavy bleeding after surgery—look exactly like those of congenital VWD [1]. Blood tests will also show low levels or poor function of the von Willebrand factor [20]. While bleeding can be frightening, it is often a manageable nuisance, though severe cases or internal bleeding require immediate medical attention.

The biggest difference lies in the long-term treatment plan. Genetic VWD is generally a lifelong condition managed by replacing the missing factor or taking medication to boost it when bleeding occurs. For AVWS, the ultimate goal is to cure or resolve the bleeding by treating the root cause [21]. For example, fixing a narrowed heart valve with surgery or treating an underlying blood cancer with medication can often completely reverse the AVWS [22][23].

Safety and Daily Management While Waiting for Treatment

Because the underlying cause of AVWS (like a heart valve issue or blood disorder) can take time to treat, you must protect yourself from bleeding complications in the meantime.

A critical safety issue for AVWS patients is medication conflict. Because many patients with AVWS also have cardiovascular conditions, they are often prescribed blood thinners, daily aspirin, or take over-the-counter NSAIDs (like ibuprofen) [24]. Combining these with a new bleeding disorder can be dangerous. You must review every medication you take with your care team immediately to ensure it is safe to continue.

If you have a bleeding emergency or need surgery while waiting for your underlying condition to be treated, doctors will use factor replacement therapies or other medications to temporarily control your bleeding [21]. However, it is important to know that in AVWS, these replacement treatments might wear off much faster than they would in genetic VWD, because your body is actively clearing or destroying the protein.

Because AVWS involves multiple systems in your body, it is highly recommended to have your care coordinated between a hematologist (blood specialist) and the specialist treating your underlying condition (like a cardiologist or oncologist).

Common questions in this guide

Can You Get an Epidural with Von Willebrand Disease?Can You Have Iron Deficiency Without Anemia in VWD?Can You Play Sports with Von Willebrand Disease?Does Stress Affect Von Willebrand Disease Test Results?How Do Antifibrinolytics Work for VWD Bleeding?How Does Type O Blood Affect Von Willebrand Disease?How Is Von Willebrand Disease Inherited?Dental Work with Von Willebrand Disease: How to PrepareIs an IUD Safe for Women with Von Willebrand Disease?What Causes Delayed Postpartum Hemorrhage in VWD?What is a DDAVP Challenge Test for Von Willebrand Disease?What Is a Heavy Period with Von Willebrand Disease?Low VWF vs. Type 1 VWD: What is the Difference?Type 2N VWD vs. Hemophilia A: What's the Difference?Does Von Willebrand Disease Affect Life Expectancy?What Pain Meds Are Safe for Von Willebrand Disease?What's in a VWD Emergency Travel Letter?When Are Nosebleeds a Sign of Von Willebrand Disease?Why Must I Restrict Fluids After DDAVP?
Can you get Von Willebrand Disease if you weren't born with it?
Yes, you can develop Acquired Von Willebrand Syndrome (AVWS) later in life. This condition causes the exact same bleeding symptoms as genetic Von Willebrand Disease but is triggered by another underlying health issue rather than genetics.
What causes Acquired Von Willebrand Syndrome?
AVWS is caused by medical conditions that destroy, absorb, or block the von Willebrand factor protein in your blood. Common triggers include heart valve issues like severe aortic stenosis, certain blood cancers, and autoimmune or thyroid conditions.
How is acquired VWD treated differently than genetic VWD?
Genetic Von Willebrand Disease requires lifelong management to control bleeding, whereas AVWS can often be resolved by treating the root cause. For example, fixing a narrowed heart valve or treating an underlying blood cancer may completely reverse the AVWS.
Is it safe to take NSAIDs or blood thinners if I have AVWS?
Combining blood thinners, daily aspirin, or NSAIDs like ibuprofen with a new bleeding disorder can be extremely dangerous. You should review all of your prescriptions and over-the-counter medications with your care team immediately to ensure they are safe to continue.
What is Heyde's syndrome?
Heyde's syndrome is a specific condition where severe aortic stenosis (a narrowing of the heart valve) causes Acquired Von Willebrand Syndrome. The mechanical stress from the heart valve destroys the von Willebrand factor, frequently leading to gastrointestinal bleeding.

Questions for Your Doctor

4 questions

  • •Given my new AVWS diagnosis, is it safe for me to continue my current dosages of aspirin, NSAIDs, or blood thinners?
  • •Who will act as the lead coordinator for my care between hematology and my other specialists (e.g., cardiology, oncology, or endocrinology)?
  • •If I have an emergency bleed or require a procedure, what is the protocol for factor replacement, and will I need larger or more frequent doses than a typical VWD patient?
  • •Are my current bleeding symptoms considered an immediate danger, or are they primarily a quality-of-life issue while we treat the underlying cause?

Questions for You

3 questions

  • •At what age did I first start noticing unusual bleeding, bruising, or prolonged nosebleeds, and did it coincide with any other new health issues?
  • •What over-the-counter medications, supplements, or prescriptions am I currently taking that I need to review with my doctor?
  • •Have I been diagnosed with any heart murmurs, blood disorders, autoimmune conditions, or thyroid issues recently?

References

References (24)
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    Same Syndrome, Different Causes and Treatment: Path to Diagnosis and Management of Two Interesting Cases of Acquired von Willebrand Syndrome.

    Monti M, Massari E, Rosetti M, et al.

    Seminars in thrombosis and hemostasis 2024; (50(2)):307-309 doi:10.1055/s-0043-1772837.

    PMID: 37640045
  2. 2

    Acquired von Willebrand syndrome during systemic mastocytosis: an analysis of 21 cases.

    Mital A, Prejzner W, Hellmann A

    Polish archives of internal medicine 2018; (128(7-8)):491-493 doi:10.20452/pamw.4295.

    PMID: 30057374
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    Treatment of the acquired von Willebrand syndrome.

    Budde U, Scheppenheim S, Dittmer R

    Expert review of hematology 2015; (8(6)):799-818 doi:10.1586/17474086.2015.1060854.

    PMID: 26577336
  4. 4

    Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage.

    Wang S, Hu Q, Chen Y, et al.

    Frontiers in pediatrics 2022; (10()):1013764 doi:10.3389/fped.2022.1013764.

    PMID: 36204663
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    Acquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management.

    Abou-Ismail MY, Rodgers GM, Bray PF, Lim MY

    Research and practice in thrombosis and haemostasis 2021; (5(2)):356-365 doi:10.1002/rth2.12481.

    PMID: 33733035
  6. 6

    von Willebrand factor disruption and continuous-flow circulatory devices.

    Proudfoot AG, Davidson SJ, Strueber M

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    PMID: 28756118
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    Inflammation, von Willebrand factor, and ADAMTS13.

    Chen J, Chung DW

    Blood 2018; (132(2)):141-147 doi:10.1182/blood-2018-02-769000.

    PMID: 29866815
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    Heyde's syndrome: a challenging case of severe aortic stenosis and gastrointestinal bleeding.

    Abouzid M, Abdelhakeem A, Elshafie S, Ghorab A

    BMJ case reports 2024; (17(4)) doi:10.1136/bcr-2023-258316.

    PMID: 38631812
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    Heyde's syndrome: a systematic review of case reports.

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    PMID: 35534046
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    Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.

    Kubicki R, Stiller B, Kroll J, et al.

    European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2019; (55(6)):1194-1201 doi:10.1093/ejcts/ezy408.

    PMID: 30590475
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    Shear-induced acquired von Willebrand syndrome: an accomplice of bleeding events in adults on extracorporeal membrane oxygenation support.

    Wang H, Li D, Chen Y, et al.

    Frontiers in cardiovascular medicine 2023; (10()):1159894 doi:10.3389/fcvm.2023.1159894.

    PMID: 37485275
  12. 12

    Practical management of the haemorrhagic complications of myeloproliferative neoplasms.

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    British journal of haematology 2022; (199(3)):313-321 doi:10.1111/bjh.18322.

    PMID: 35724983
  13. 13

    Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias.

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    Annals of hematology 2016; (95(6)):945-57 doi:10.1007/s00277-016-2650-x.

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    Acquired von Willebrand Syndrome.

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    PMID: 34391604
  15. 15

    Lenalidomide as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy.

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    Increased cleavage of von Willebrand factor by ADAMTS13 may contribute strongly to acquired von Willebrand syndrome development in patients with essential thrombocythemia.

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    Cryptic non-canonical splice site activation is part of the mechanism that abolishes multimer organization in the c.2269_2270del von Willebrand factor.

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    Haematologica 2020; (105(4)):1120-1128 doi:10.3324/haematol.2019.222679.

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    Is Extracorporeal CO2 Removal Really "Safe" and "Less" Invasive? Observation of Blood Injury and Coagulation Impairment during ECCO2R.

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    Acquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome.

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  21. 21

    Successful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy : Breaking a dangerous bond.

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This page provides educational information about Acquired Von Willebrand Syndrome (AVWS) and late-onset bleeding symptoms. Always consult your hematologist or primary care provider to evaluate new bleeding symptoms and before changing any medications.

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