Haemophilia

Summary of Haemophilias

Haemophilia definition: condition causing bleeding

Genetic 

• Haemophilia A & B

• Von Willebrand Disease

Acquired

• Acquired Haemophilia

Haemophilia A & B

• Haemophilia A- factor 8, 

• Haemophilia B- factor 9 (Christmas disease)

• X linked recessive

• 2nd most common bleeding disorder (Von Willebrand Disease)

• Presents with coagulopathic bleeding: hemarthrosis, normal PT and APTT

Acquired Haemophilia

• Associated with anti-factor VIII IgG antibodies

• Cause

  • Idiopathic (majority)

  • Secondary:

    •  Rheumatoid Arthritis, IBD

    • Drugs- phenytoin

• Investigations: prolonged PT & APTT

• Management: 

  • Low level of antibodies: factor VIII replacement

  • Bleeding: corticosteroids +- ciclosporin 

  • Refractory bleeding: recombinant factor VII

Page written in 2024.