Management of Atypical Presentations of Mild to Moderate Von Willebrand Disease

Clinical Presentation and Diagnosis

• Acquired von Willebrand syndrome (AVWS) may present with atypical symptoms and should be considered in patients with abnormal VWF test results and bleeding symptoms without a personal/family history consistent with hereditary VWD 1
• In aortic stenosis, impaired platelet function and decreased levels of von Willebrand factor can be demonstrated, with severity correlating with the degree of stenosis 2, 3
• Acquired VWD in aortic stenosis is associated with clinical bleeding (epistaxis or ecchymoses) in approximately 20% of patients 3, 4
• Initial evaluation should include assessment of personal and family bleeding history, with specific attention to mucocutaneous bleeding patterns 6
• Laboratory testing should include complete blood count (CBC), prothrombin time (PT), activated partial thromboplastin time (aPTT), VWF antigen (VWF:Ag), VWF activity (VWF:RCo), and factor VIII activity 1

Management and Treatment

• Treatment should target the underlying condition (e.g., aortic stenosis) 3, 4
• In myeloproliferative disorders with acquired VWD, aspirin should be used with caution due to increased bleeding risk 7
• For surgical procedures, target VWF activity level should be ≥50 IU/dL 5
• VWF activity should be maintained >50 IU/dL while epidural catheter remains in place for neuraxial procedures 5

Special Considerations

• In aortic stenosis-associated VWD, valve replacement may resolve the coagulation abnormality except when the prosthetic valve area is small for patient size (less than 0.8 cm² per m²) 3, 4
• For persistent mucosal bleeding despite adequate FVIII levels, consider adjunctive treatments like tranexamic acid 5
• In myeloproliferative neoplasms, cytoreductive therapy (hydroxyurea or anagrelide) with aspirin is recommended for high-risk disease, but aspirin should be used cautiously in patients with acquired VWD 7