Typical Progression of Hereditary von Willebrand Disease
Hereditary von Willebrand disease is a lifelong, non-progressive bleeding disorder where symptoms remain relatively stable throughout life, with severity determined by VWD type at birth rather than worsening over time.
Disease Stability Over Time
The clinical course of hereditary VWD does not follow a progressive deterioration pattern. The bleeding phenotype established in childhood typically persists throughout life without significant worsening of the underlying VWF deficiency 1. The severity is determined by the specific type and degree of VWF deficiency or dysfunction present from birth 2.
Type-Specific Clinical Patterns
Type 1 VWD (75% of cases)
- Mild to moderate bleeding symptoms that remain consistent over time 1
- Mucocutaneous bleeding (epistaxis, easy bruising, menorrhagia) is typical 3, 2
- Bleeding after dental extraction is the most frequent postoperative complication 3
- Spontaneous hemarthroses or deep tissue hematomas are rare because Factor VIII levels remain relatively preserved 3
- Post-delivery bleeding is uncommon because VWF levels normalize by the end of pregnancy 3
Type 2 VWD (Most remaining cases)
- Moderate bleeding severity that remains stable 1
- Clinical manifestations similar to Type 1 but may be more pronounced depending on the specific subtype (2A, 2B, 2M, 2N) 1, 2
- Bleeding patterns established early in life persist without progression 3
Type 3 VWD (Rare: 1 in 1,000)
- Severe, life-threatening bleeding potential from birth that resembles hemophilia 1, 3
- May include spontaneous hemarthroses and deep tissue hematomas due to severely reduced Factor VIII 3
- Requires prophylactic treatment before any surgical procedure 3
- No spontaneous bleeding usually occurs at birth despite severe deficiency 4
Age-Related Considerations
Childhood Presentation
- Bleeding history typically evident since childhood 4
- Diagnosis most commonly occurs in young adults (83% of cases), predominantly in women (57%) due to menorrhagia bringing them to medical attention 3
Pregnancy Effects
- VWF and Factor VIII levels increase during pregnancy, often normalizing by the third trimester in Type 1 VWD 3
- This temporary improvement does not represent disease progression but rather physiological response to pregnancy 3
Lifelong Stability
- The disorder does not worsen with aging in the absence of acquired factors 1
- External variables (blood type, exercise, thyroid hormones, estrogens, aging) may influence VWF levels but do not represent true disease progression 2
Important Clinical Caveats
The majority of VWD patients (82% in prospective studies) do not experience bleeding episodes in a given year, emphasizing the non-progressive and often mild nature of the condition 3. When bleeding does occur, mucosal bleeding (64%) is far more common than hematomas or hemarthroses (15%), and most patients (63%) never require transfusions 3.
Hereditary VWD must be distinguished from acquired von Willebrand syndrome (AVWS), which can develop later in life secondary to underlying medical conditions and may worsen as the causative condition progresses 5. AVWS is characterized by absence of personal and family bleeding history and association with lymphoproliferative disorders, cardiovascular conditions, or autoimmune diseases 5.