From the Guidelines
Von Willebrand disease should be managed with desmopressin (DDAVP) as the first-line treatment, given as a nasal spray at 150-300 mcg or intravenously at 0.3 mcg/kg, as it is effective in releasing stored VWF from blood vessel walls and improving hemostasis 1.
Treatment Options
The treatment of Von Willebrand disease depends on the type and severity of the condition. The main treatment options include:
- Desmopressin (DDAVP): a synthetic hormone that stimulates the release of VWF from blood vessel walls
- VWF replacement therapy: products like Humate-P, Wilate, or Vonvendi may be necessary for more severe cases or when DDAVP is ineffective
- Antifibrinolytic medications: tranexamic acid or aminocaproic acid can help stabilize clots
- Topical agents: fibrin glue may be sufficient for minor bleeding
Patient Management
Patients with Von Willebrand disease should:
- Avoid aspirin and NSAIDs, which can worsen bleeding tendencies
- Wear medical alert identification and inform healthcare providers before procedures
- Undergo regular monitoring of VWF levels to guide long-term management
- Consider hormonal contraceptives for women with heavy menstrual bleeding
Severity-Based Treatment
The severity of Von Willebrand disease can range from mild to severe, and treatment should be tailored accordingly. For example:
- Type 1 VWD: DDAVP may be sufficient for mild cases
- Type 2 VWD: VWF replacement therapy may be necessary for more severe cases
- Type 3 VWD: VWF replacement therapy is often required due to the severe deficiency of VWF It is essential to note that the treatment of Von Willebrand disease should be individualized based on the patient's specific needs and circumstances, and that the most recent and highest-quality study should be prioritized when making treatment decisions 1.
From the Research
Von Willebrand Deficiency Overview
- Von Willebrand disease (VWD) is an inherited condition characterized by deficiency of von Willebrand factor, which is essential in hemostasis 2.
- The disease has three major subtypes: type 1 (partial quantitative deficiency), type 2 (qualitative deficiency), and type 3 (virtually complete deficiency) 2.
Diagnosis and Management
- Diagnosis is usually made by reviewing the patient's personal and family history of bleeding and by clinical evaluation for more common reasons for bleeding, supplemented with laboratory tests 2.
- Assessment may be used to determine bleeding risk before surgery and other invasive procedures, and to diagnose reasons for unexplained hemorrhaging 2.
- Treatment is tailored to the subtype of the disease: increasing plasma concentration of von Willebrand factor by releasing endogenous stores with desmopressin or replacing nonexistent or ineffective von Willebrand factor by using human plasma-derived, viral-inactivated concentrates 2.
Treatment Options
- Desmopressin (DDAVP) is the treatment of choice for type 1 VWD because it can induce release of normal VWF from cellular compartments 3.
- DDAVP is not effective in VWD type 3 and in severe forms of VWD 1 and 2 3.
- Plasma-derived VWF/FVIII products may also be substituted exogenously 4.
- Treatment with plasma-derived or recombinant VWF concentrates without FVIII is also possible 4.
Replacement Therapy
- Replacement therapy may be indicated in the case of spontaneous bleeding, surgical interventions and injuries/trauma or as a prophylaxis of spontaneous bleeding episodes 5.
- Dual VWF/FVIII concentrates can be beneficial in the case of low FVIII level, while repeated dosing may lead to very high FVIII levels, with a potential thrombogenic effect in individual VWD patients 5.
- An excessive FVIII:C increase can be limited by using a VWF product with a low level of FVIII, achieving a haemostatic adequate FVIII:C increase after 6 to 12 hours 5.