What is the recommended management for a 65-year-old patient with mild to moderate von Willebrand factor deficiency?

Management of Mild to Moderate von Willebrand Factor Deficiency in a 65-Year-Old Patient

For a 65-year-old patient with mild to moderate von Willebrand factor deficiency, desmopressin (DDAVP) is the recommended first-line treatment, as it effectively promotes the release of von Willebrand factor and Factor VIII from the endothelium. 1

Diagnostic Considerations

The patient's laboratory findings show:

• von Willebrand factor activity of 1.26 (elevated)
• Antigen activity ratio of 0.7

These findings require careful interpretation:

• VWF is an acute phase reactant that can be falsely elevated in response to stress, inflammation, age, or other clinical conditions 2
• A VWF:RCo/VWF:Ag ratio <0.5-0.7 may indicate a qualitative defect (Type 2 VWD) 2
• Age-related increases in VWF levels are common, which may mask underlying VWD 2, 3

Treatment Algorithm

First-Line Treatment

• Desmopressin (DDAVP) is indicated for patients with mild to moderate von Willebrand disease (Type I) with factor VIII levels greater than 5% 1
• DDAVP promotes the release of VWF and FVIII from the endothelium 4

When to Use DDAVP

1. For maintenance of hemostasis during surgical procedures and postoperatively
2. To reduce bleeding with episodes of spontaneous or traumatic injuries
3. For mucosal bleeding management 1

Dosing

• The recommended dosage is 0.3 mcg/kg administered intravenously 1
• Fluid restriction should be initiated during treatment to prevent hyponatremia 1

Monitoring

• Monitor serum sodium, bleeding time, factor VIII coagulant activity, ristocetin cofactor activity, and von Willebrand antigen to ensure adequate levels are achieved 1
• Measure serum sodium within 7 days and approximately 1 month after initiating therapy 1

Alternative Treatment Options

For patients who do not respond to DDAVP or have contraindications:

• VWF-containing factor concentrates are indicated for:
  • Severe forms of Type 1 VWD
  • Type 2B VWD
  • When DDAVP is ineffective 4, 2

Special Considerations for Older Patients

1. Thrombotic Risk: Balance hemostasis with thrombosis risk, especially in older patients 2

2. Hyponatremia Risk: Older patients are at increased risk of hyponatremia with DDAVP treatment 1

   • More frequent monitoring of serum sodium is required in patients ≥65 years 1
   • DDAVP may need to be temporarily or permanently discontinued if hyponatremia occurs 1

3. Comorbidities: Assess for conditions that may increase risk:

   • Excessive fluid intake
   • Illnesses causing fluid or electrolyte imbalances
   • Use of loop diuretics or systemic/inhaled glucocorticoids 1

Perioperative Management

For surgical procedures:

• Target VWF activity level of ≥50 IU/dL 4, 2
• For major procedures, maintain VWF:RCo >50 IU/dL for 72 hours post-surgery 2
• Inform the surgical team about bleeding risk and consider meticulous surgical technique 2

Common Pitfalls to Avoid

1. Relying on a single VWF measurement: VWF levels can vary and may need to be repeated up to 3 times for reliable results 2

2. Ignoring age-related increases: The patient's elevated VWF activity (1.26) may be due to age-related increases, potentially masking an underlying deficiency 3

3. Overlooking qualitative defects: The antigen activity ratio of 0.7 suggests a possible qualitative defect despite normal or elevated VWF levels 2

4. Medication interactions: Avoid concomitant use of aspirin or other platelet antiaggregants 2

By following this management approach, you can effectively address the mild to moderate von Willebrand factor deficiency in this 65-year-old patient while minimizing risks associated with treatment.