How Serious Is 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.

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.

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 

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. 

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 

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

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.

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.