Von Willebrand Disease

Exam Cram

• Most common inherited bleeding disorder (haemophilia)

• MoA: low levels of Von Willebrand Factor is unable to activate clotting ability of factor VIII

• Presents with platelet bleeding: bleeding gums, menorrhagia, bleeding ++ after minor procedures

• Management: desmopressin, tranexamic acid

Outline

Background

• Type 1 (70%) is caused by deficiency in Von Willebrand Factor 

• Inheritance: autosomal dominant

Assessment

Presentation

• Bleeding post procedure, e.g. wisdom tooth removal

• Unexplained menorrhagia

• Mucosal bleeding: epistaxis

• Family history of bleeding issues

Investigations

• APTT usually prolonged but can be normal

• Normal PT

Diagnosis 

Diagnostic criteria: clinical, no single test

Differentials: 

• Haemophilia presents with haemarthrosis and more usually has a raised APTT

• Von Willebrand is more common- presents with bleeding gums, can have prolonged APTT or not

Management

• Desmopressin

• Tranexamic Acid

Resources and References

1. https://www.cdc.gov/von-willebrand/diagnosis/index.html

Written 2024.