How Serious Is von Willebrand Disease?

What Is von Willebrand Disease?

von Willebrand Disease Test
For the most part, von Willebrand disease is a mild, manageable bleeding disorder. Severe bleeding usually only occurs due to trauma or surgery.

Von Willebrand disease is a common bleeding disorder caused by a deficiency or dysfunction of a protein called von Willebrand factor (vWF). 

Von Willebrand disease has three major categories:

  • Type 1 – Partial quantitative vWF deficiency - the most common form, affects about 75% of patients. People with this type do not produce enough von Willebrand factor or the vWF is removed abnormally fast from the blood.
  • Type 2 – Qualitative vWF deficiency - second-most common form. People with this form produce abnormal vWF, and the factor functions abnormally.
  • Type 3 - Total vWF deficiency - least common form. People with this form do not make vWF or it is removed extremely quickly from the bloodstream. Type 3 is detected early in life because it causes the most severe bleeding symptoms.

What Are Symptoms of von Willebrand Disease?

Symptoms of von Willebrand disease include:

  • Nosebleeds
  • Bruises (hematomas)
  • Prolonged bleeding from minor wounds
  • Heavy bleeding in the mouth, particularly after tooth extraction or other oral surgery, such as tonsillectomy and adenoidectomy
  • Excessive menstrual bleeding 
  • Severe bleeding after major surgery (less common) or delayed bleeding that may occur several weeks after surgery 
  • Worsening of bleeding symptoms after taking aspirin 
  • Improvement of bleeding symptoms with use of oral contraceptives 

Symptoms of von Willebrand disease specific to children include:

  • Umbilical stump bleeding 
  • Blood accumulation between the scalp and skull (cephalohematoma)
  • Bruising on the cheek 
  • Bleeding in the conjunctiva of the eyes
  • Post-circumcision bleeding 
  • Post-venipuncture bleeding 

What Causes von Willebrand Disease?

In most cases, von Willebrand disease is a genetic, inherited condition. 

Rarely, von Willebrand disease may be acquired and is a result of the development of antibodies to von Willebrand factor in the blood. Acquired von Willebrand disease may result from certain diseases:

  • Lymphoproliferative disorders such as leukemia, lymphomas, myelomas
  • Cardiovascular diseases
  • Myeloproliferative diseases which are rare blood cancers

Acquired von Willebrand disease often resolves when the underlying diseases are treated. 


How Is von Willebrand Disease Diagnosed?

Von Willebrand disease is diagnosed with a bleeding history such as if you bruise easily or have frequent nosebleeds, and a family history, since the condition tends to run in families. 

Lab tests that may be used to diagnose von Willebrand disease include:

  • von Willebrand factor (VWF) antigen
  • von Willebrand factor activity test – a commonly used vWF activity test is called the Ristocetin cofactor test (VWF:RCo)
  • Factor VIII (factor eight) activity 
  • Prothrombin time (PT) 
  • Activated partial thromboplastin time (aPTT) 

Additional testing may be indicated to confirm the specific subtype of von Willebrand disease and may include:

  • vWF collagen-binding activity 
  • Low-dose ristocetin vWF-platelet binding 
  • FVIII-vWF binding 
  • vWF multimer analysis 
  • vWF propeptide antigen 


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What Is the Treatment for von Willebrand Disease?

The main treatments for von Willebrand disease (vWD) include:

  • Desmopressin (DDAVP
  • Recombinant von Willebrand factor (rVWF) – used as needed to treat minor or major bleeding episodes in adults with vWD 
  • von Willebrand factor/factor VIII (vWF/FVIII) concentrates used to treat bleeds and for surgical bleeding prevention when DDAVP is ineffective or contraindicated 
  • Antifibrinolytic drugs (i.e., aminocaproic acid, tranexamic acid) to treat mild mucocutaneous bleeding
  • Topical agents (e.g., fibrin sealants) as optional adjunctive therapy for dental surgery and surface wound bleeding unresponsive to drugs and concentrates
  • Platelet transfusions 
  • Birth control pills or a progestin-releasing intrauterine device (IUD) for women with heavy menstrual bleeding

What Are Complications of von Willebrand Disease?

Complications of desmopressin (DDAVP) treatment for von Willebrand disease (vWD) type 1 include fluid retention and low blood sodium (hyponatremia).

Patients with type 2B vWD disease may experience low blood platelets (thrombocytopenia).

Complications of Willebrand disease (vWD) type 3 include alloantibody formation which can put patients at increased risk for life-endangering anaphylactic reactions to vWF-FVIII preparations. 

Complications of von Willebrand disease in pregnancy may include bleeding problems.


What Is the Life Expectancy for von Willebrand Disease?

For the most part, von Willebrand disease is a mild, manageable bleeding disorder. Severe bleeding usually only occurs due to trauma or surgery. 

In patients with vWD types 2 and 3, bleeding episodes can be severe and even life-threatening.

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