What is the management approach for patients with von Willebrand's disease and gastrointestinal (GI) angiodysplasia?

Management of von Willebrand Disease with GI Angiodysplasia

Diagnosis and Assessment

For patients with von Willebrand disease (VWD) and suspected GI angiodysplasia, video capsule endoscopy (VCE) should be used on top of conventional endoscopy to improve diagnosis and outcome prediction. 1

The diagnostic approach should include:

• Complete laboratory evaluation including VWF antigen (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), and factor VIII coagulant activity (FVIII) 2
• Calculate VWF:RCo to VWF:Ag ratio (ratio below 0.5-0.7 suggests Type 2 VWD) 2
• Comprehensive endoscopic evaluation:
  • Conventional endoscopy (upper and lower GI tract)
  • Video capsule endoscopy (VCE) - significantly improves detection of small bowel angiodysplasia 1
  • Multiple radiologic and endoscopic investigations may be required as diagnosis remains challenging 3

Treatment Algorithm

Acute Management

1. VWF-containing factor concentrates:

   • First-line treatment for acute bleeding episodes 2
   • Target VWF activity level of ≥50 IU/dL 2
   • Monitor VWF:RCo and FVIII:C levels at 12-24 hours post-treatment 2

2. Endoscopic treatment:

   • Argon plasma coagulation for accessible lesions 1
   • Note that patients with small bowel angiodysplasia have higher recurrence rates even after endoscopic treatment 1

Long-term Management

1. Prophylactic VWF concentrate therapy:

   • Most effective method for preventing recurrent GI bleeding in VWD patients with angiodysplasia 4
   • Particularly important for Type 2B, Type 3, and severe forms of Type 1 and 2 VWD 2

2. Hormonal therapy:

   • Estrogen/progesterone therapy can be considered as an alternative to repeated blood product transfusions or extensive surgical resection 5
   • May be effective in preventing recurrent bleeding for extended periods 5

3. Desmopressin (DDAVP):

   • Consider only in patients with mild to moderate Type 1 VWD 2
   • Not recommended for Type 2B, Type 3, or severe Type 1 VWD 2
   • Often ineffective for angiodysplasia-related bleeding 6

Special Considerations

• Patients with VWD and angiodysplasia have significantly higher rates of GI bleeding recurrence compared to those without angiodysplasia 1
• Small bowel localizations of angiodysplasia are associated with worse prognosis and higher recurrence rates 1
• Balance hemostasis with thrombosis risk, especially in older patients 2
• Avoid concomitant use of aspirin or other platelet antiaggregants with metoclopramide as it may worsen platelet dysfunction 2
• Consultation with a hematologist is essential for optimal management 2

Monitoring and Follow-up

• Document response to interventions to guide future management 2
• Regular follow-up to assess for recurrent bleeding
• Repeat endoscopic evaluations may be necessary for patients with recurrent bleeding
• Consider prophylactic therapy adjustment based on bleeding pattern