Table of ContentsView AllTable of ContentsWhat Is It?CausesTypesSymptomsDiagnosisTreatmentPreventionWhen to See a Provider
Table of ContentsView All
View All
Table of Contents
What Is It?
Causes
Types
Symptoms
Diagnosis
Treatment
Prevention
When to See a Provider
Von Willebranddisease (VWD) is a disorder associated with bleeding more than usual. Individuals with VWD have low levels of or a defective von Willebrand factor (VWF), a vital protein in blood clotting.
VWD is nearly always inherited (passed down from parent to child) and lifelong. Even though most people are born with VWD, it’s often not diagnosed until adulthood, as symptoms tend to be mild.Rarely. it can be acquired. In acquired cases, VWD will resolve when the underlying condition has been treated.
This article reviews what it means to have von Willebrand disease, including its cause, types, and symptoms, and how it’s diagnosed and managed.
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What Happens If You Have Von Willebrand Disease?
The impaired blood clotting process in VWD leads to excessive bleeding, manifesting as spontaneousnosebleeds,easy bruising, and/or heavy menstrual periods.
Longer-than-normal bleeding after a minor cut, dental procedure, surgery, or childbirth is also common in people with VWD. In some cases,anemiadevelops as a result of excessive bleeding.
Very rarely is the bleeding in VWD severe or life-threatening, such as when it affects internal organs like the stomach or intestines.
How Common Is VWD?VWD is estimated to affect around 1% of the general population, approximately 1 in 100 people.
How Common Is VWD?
VWD is estimated to affect around 1% of the general population, approximately 1 in 100 people.
What Causes Von Willebrand Disease?
In most cases (the exception is acquired VWD), the disease is inherited, meaning a person is born with it.
Genemutations(alteration inDNAsequencing) that cause either a deficiency of VWF or a malfunctioning VWF result in von Willebrand disease.
VWF has these main functions:
What Are Coagulopathy Disorders?
Von Willebrand Disease Types
There are three main types of von Willebrand disease, varying in severity and inheritance pattern.
Type 1
Type 1 affects approximately 85% of individuals with VWD in the United States. It is inherited in an autosomal dominant pattern.
Inheritance PatternsAutosomal dominant inheritancemeans only one copy of the altered VWF gene needs to be passed on from parent to child to cause the disease, and the genes are carried on the autosomal chromosomes (other than the sex chromosomes X or Y, so there usually is no difference in occurrence based on sex).Autosomal recessiverequires inheriting two copies of the altered gene (one from each parent).
Inheritance Patterns
Autosomal dominant inheritancemeans only one copy of the altered VWF gene needs to be passed on from parent to child to cause the disease, and the genes are carried on the autosomal chromosomes (other than the sex chromosomes X or Y, so there usually is no difference in occurrence based on sex).Autosomal recessiverequires inheriting two copies of the altered gene (one from each parent).
Bleeding symptoms in type 1 VWD tend to be mild to moderate.
Type 2
Type 2 VWD occurs as a result of a defective VWF protein. Bleeding symptoms are generally more severe than they are with type 1 VWD.
Knowing what subtype you are is essential for guiding treatment decisions.
The four type 2 subtypes are:
Regarding inheritance patterns, Type 2B and 2M show autosomal dominant inheritance, and Type 2N shows autosomal recessive inheritance. Type 2A may be inherited as autosomal dominant or recessive.
Type 3
Type 3 is inherited in an autosomal recessive pattern and is the rarest type of VWD, occurring in less than 1% of cases in the United States.
Type 3 VWD is usually diagnosed early in life as a person produces virtually no VWF, resulting in severe bleeding symptoms.
Acquired
Acquired VWD occurs when a person has a deficiency of or dysfunctional VWF because of an underlying health condition—not because of inherited genetic changes.
Diseases known to lead to acquired VWD potentially include:
Many medications have also been linked to acquired VWD, such as the anti-seizure drug Depakote (valproic acid) and the antibiotic Cipro (ciprofloxacin).
Von Willebrand Symptoms
Symptoms of von Willebrand disease vary based on disease type, age, and whether a person menstruates.
The most common symptoms of von Willebrand disease in decreasing frequency include:
Are Blood Clots Normal During a Period?
Other symptoms (more common in type 3 VWD) include:
What Causes Random Bruising?
How Is Von Willebrand Disease Diagnosed?
There is no single test to diagnose von Willebrand disease. Instead, a healthcare provider considers a person’s symptoms, their family history of bleeding, and the results of various blood tests.
These blood tests include:
In VWD type 1 (most common), the CBC is normal, except possibly for anemia, which would show up as low hemoglobin. There is a mild reduction in von Willebrand factor antigen and the VWF: Rco test. Factor VIII clotting activity is mildly decreased or normal.
Additional tests are performed to help define the VWD subtype. As an example, thevon Willebrand factor multimer analysismeasures the size and structure of VWF and is particularly useful for identifying vWD types 2A and 2B.
Von Willebrand Disease Treatment
The treatment of von Willebrand disease depends on the type, symptoms, and severity of bleeding.
Such treatments include:
Treating acquired VWF entails managing the underlying condition or stopping the offending medication if applicable.
Can You Prevent Von Willebrand Disease?
You cannot prevent von Willebrand disease, as the disease is inherited in most cases. The good news is that while not preventable, the condition can be managed well.
Remain ProactiveTo optimize care in a medical emergency, wear a medical identification bracelet or necklace, noting what type of VWD you have.
Remain Proactive
To optimize care in a medical emergency, wear a medical identification bracelet or necklace, noting what type of VWD you have.
When to Contact a Healthcare Provider
If you have a history of increased bleeding, especially nosebleeds, abnormal bruising, heavy or prolonged menstrual periods, or extended bleeding after dental procedures or childbirth, bring this to the attention of a healthcare provider.
A family history of VWD or other bleeding disorders suggestive of VWD is another reason for seeing a healthcare provider.
When seeing the healthcare provider, they may utilize a bleeding assessment tool (BAT) to determine whether you need blood testing for VWD.
Seek Emergency Medical AttentionGo to your nearest emergency room or call 911 if you cannot control your bleeding with direct pressure, are losing a large amount of blood, or have severe joint swelling and pain.
Seek Emergency Medical Attention
Go to your nearest emergency room or call 911 if you cannot control your bleeding with direct pressure, are losing a large amount of blood, or have severe joint swelling and pain.
Summary
Von Willebrand disease (VWD) causes excessive bleeding due to a defective or reduced amount of von Willebrand factor, a protein involved in blood clotting. This bleeding disease is nearly always inherited and commonly linked to heavy or prolonged menstrual bleeding, spontaneous nosebleeds, easy bruising, and extended bleeding after a minor cut or childbirth.
The diagnosis of VWD requires reviewing symptoms with a healthcare provider and undergoing various blood tests. Even though VWD is not preventable or curable, it can be managed. Specific therapies depend on a person’s VWD type (there are three main types) and bleeding symptoms and severity.
13 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Du P, Bergamasco A, Moride Y, et al.Von Willebrand disease epidemiology, burden of illness and management: a systematic review.J Blood Med. 2023;14:189-208. doi:10.2147/JBM.S389241Franchini M, Mannucci PM.Acquired von Willebrand syndrome: focused for hematologists.Haematologica. 2020;105(8):2032-2037. doi:10.3324/haematol.2020.255117Palta S, Saroa R, Palta A.Overview of the coagulation system.Indian J Anaesth. 2014;58(5):515-523. doi:10.4103/0019-5049.144643Heijdra JM, Cnossen MH, Leebeek FWG.Current and emerging options for the management of inherited von Willebrand disease.Drugs. 2017;77(14):1531-1547. doi:10.1007/s40265-017-0793-2Flood VH, Abshire TC, Christopherson PA, et al.Von Willebrand disease in the United States: perspective from the Zimmerman program.Ann Blood. 2018;3:7. doi:10.21037/aob.2017.12.05Centers for Disease Control and Prevention.What is von Willebrand disease?Mandava M, Bergmann S, Lazarchick J.A novel case of compound heterozygous type 3 von Willebrand disease.Ann Clin Lab Sci. 2019;49(3):393-394Owari M, Harada-Shirado K, Togawa R, et al.Acquired von Willebrand syndrome in a patient with multiple comorbidities, Including MALT lymphoma with IgA monoclonal gammopathy and hyperviscosity syndrome.Intern Med. 2023;62(4):605-611. doi:10.2169/internalmedicine.9815-22Chapin J.Von Willebrand disease in the elderly: clinical perspectives.Clin Interv Aging. 2018;13:1531-1541. doi:10.2147/CIA.S136931Weyand AC, Flood VH.Von Willebrand disease: current status of diagnosis and danagement.Hematol Oncol Clin North Am. 2021;35(6):1085-1101. doi:10.1016/j.hoc.2021.07.004Roberts JC, Flood VH.Laboratory diagnosis of von Willebrand disease.Int J Lab Hematol. 2015;37 Suppl 1(Suppl 1):11-7. doi:10.1111/ijlh.12345Castaman G.How I treat von Willebrand disease.Thromb Res. 2020;196:618-625. doi:10.1016/j.thromres.2020.07.051Elbatarny M, Mollah S, Grabell J, et al.Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project.Haemophilia. 2014;20(6):831-835. doi:10.1111/hae.12503
13 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Du P, Bergamasco A, Moride Y, et al.Von Willebrand disease epidemiology, burden of illness and management: a systematic review.J Blood Med. 2023;14:189-208. doi:10.2147/JBM.S389241Franchini M, Mannucci PM.Acquired von Willebrand syndrome: focused for hematologists.Haematologica. 2020;105(8):2032-2037. doi:10.3324/haematol.2020.255117Palta S, Saroa R, Palta A.Overview of the coagulation system.Indian J Anaesth. 2014;58(5):515-523. doi:10.4103/0019-5049.144643Heijdra JM, Cnossen MH, Leebeek FWG.Current and emerging options for the management of inherited von Willebrand disease.Drugs. 2017;77(14):1531-1547. doi:10.1007/s40265-017-0793-2Flood VH, Abshire TC, Christopherson PA, et al.Von Willebrand disease in the United States: perspective from the Zimmerman program.Ann Blood. 2018;3:7. doi:10.21037/aob.2017.12.05Centers for Disease Control and Prevention.What is von Willebrand disease?Mandava M, Bergmann S, Lazarchick J.A novel case of compound heterozygous type 3 von Willebrand disease.Ann Clin Lab Sci. 2019;49(3):393-394Owari M, Harada-Shirado K, Togawa R, et al.Acquired von Willebrand syndrome in a patient with multiple comorbidities, Including MALT lymphoma with IgA monoclonal gammopathy and hyperviscosity syndrome.Intern Med. 2023;62(4):605-611. doi:10.2169/internalmedicine.9815-22Chapin J.Von Willebrand disease in the elderly: clinical perspectives.Clin Interv Aging. 2018;13:1531-1541. doi:10.2147/CIA.S136931Weyand AC, Flood VH.Von Willebrand disease: current status of diagnosis and danagement.Hematol Oncol Clin North Am. 2021;35(6):1085-1101. doi:10.1016/j.hoc.2021.07.004Roberts JC, Flood VH.Laboratory diagnosis of von Willebrand disease.Int J Lab Hematol. 2015;37 Suppl 1(Suppl 1):11-7. doi:10.1111/ijlh.12345Castaman G.How I treat von Willebrand disease.Thromb Res. 2020;196:618-625. doi:10.1016/j.thromres.2020.07.051Elbatarny M, Mollah S, Grabell J, et al.Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project.Haemophilia. 2014;20(6):831-835. doi:10.1111/hae.12503
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Du P, Bergamasco A, Moride Y, et al.Von Willebrand disease epidemiology, burden of illness and management: a systematic review.J Blood Med. 2023;14:189-208. doi:10.2147/JBM.S389241Franchini M, Mannucci PM.Acquired von Willebrand syndrome: focused for hematologists.Haematologica. 2020;105(8):2032-2037. doi:10.3324/haematol.2020.255117Palta S, Saroa R, Palta A.Overview of the coagulation system.Indian J Anaesth. 2014;58(5):515-523. doi:10.4103/0019-5049.144643Heijdra JM, Cnossen MH, Leebeek FWG.Current and emerging options for the management of inherited von Willebrand disease.Drugs. 2017;77(14):1531-1547. doi:10.1007/s40265-017-0793-2Flood VH, Abshire TC, Christopherson PA, et al.Von Willebrand disease in the United States: perspective from the Zimmerman program.Ann Blood. 2018;3:7. doi:10.21037/aob.2017.12.05Centers for Disease Control and Prevention.What is von Willebrand disease?Mandava M, Bergmann S, Lazarchick J.A novel case of compound heterozygous type 3 von Willebrand disease.Ann Clin Lab Sci. 2019;49(3):393-394Owari M, Harada-Shirado K, Togawa R, et al.Acquired von Willebrand syndrome in a patient with multiple comorbidities, Including MALT lymphoma with IgA monoclonal gammopathy and hyperviscosity syndrome.Intern Med. 2023;62(4):605-611. doi:10.2169/internalmedicine.9815-22Chapin J.Von Willebrand disease in the elderly: clinical perspectives.Clin Interv Aging. 2018;13:1531-1541. doi:10.2147/CIA.S136931Weyand AC, Flood VH.Von Willebrand disease: current status of diagnosis and danagement.Hematol Oncol Clin North Am. 2021;35(6):1085-1101. doi:10.1016/j.hoc.2021.07.004Roberts JC, Flood VH.Laboratory diagnosis of von Willebrand disease.Int J Lab Hematol. 2015;37 Suppl 1(Suppl 1):11-7. doi:10.1111/ijlh.12345Castaman G.How I treat von Willebrand disease.Thromb Res. 2020;196:618-625. doi:10.1016/j.thromres.2020.07.051Elbatarny M, Mollah S, Grabell J, et al.Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project.Haemophilia. 2014;20(6):831-835. doi:10.1111/hae.12503
Du P, Bergamasco A, Moride Y, et al.Von Willebrand disease epidemiology, burden of illness and management: a systematic review.J Blood Med. 2023;14:189-208. doi:10.2147/JBM.S389241
Franchini M, Mannucci PM.Acquired von Willebrand syndrome: focused for hematologists.Haematologica. 2020;105(8):2032-2037. doi:10.3324/haematol.2020.255117
Palta S, Saroa R, Palta A.Overview of the coagulation system.Indian J Anaesth. 2014;58(5):515-523. doi:10.4103/0019-5049.144643
Heijdra JM, Cnossen MH, Leebeek FWG.Current and emerging options for the management of inherited von Willebrand disease.Drugs. 2017;77(14):1531-1547. doi:10.1007/s40265-017-0793-2
Flood VH, Abshire TC, Christopherson PA, et al.Von Willebrand disease in the United States: perspective from the Zimmerman program.Ann Blood. 2018;3:7. doi:10.21037/aob.2017.12.05
Centers for Disease Control and Prevention.What is von Willebrand disease?
Mandava M, Bergmann S, Lazarchick J.A novel case of compound heterozygous type 3 von Willebrand disease.Ann Clin Lab Sci. 2019;49(3):393-394
Owari M, Harada-Shirado K, Togawa R, et al.Acquired von Willebrand syndrome in a patient with multiple comorbidities, Including MALT lymphoma with IgA monoclonal gammopathy and hyperviscosity syndrome.Intern Med. 2023;62(4):605-611. doi:10.2169/internalmedicine.9815-22
Chapin J.Von Willebrand disease in the elderly: clinical perspectives.Clin Interv Aging. 2018;13:1531-1541. doi:10.2147/CIA.S136931
Weyand AC, Flood VH.Von Willebrand disease: current status of diagnosis and danagement.Hematol Oncol Clin North Am. 2021;35(6):1085-1101. doi:10.1016/j.hoc.2021.07.004
Roberts JC, Flood VH.Laboratory diagnosis of von Willebrand disease.Int J Lab Hematol. 2015;37 Suppl 1(Suppl 1):11-7. doi:10.1111/ijlh.12345
Castaman G.How I treat von Willebrand disease.Thromb Res. 2020;196:618-625. doi:10.1016/j.thromres.2020.07.051
Elbatarny M, Mollah S, Grabell J, et al.Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project.Haemophilia. 2014;20(6):831-835. doi:10.1111/hae.12503
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