Haemophilia A

  • Factor VIII deficiency

  • X-linked

Epidemiology

  • 50 cases / million

Clinical Features

  • range

  • severe bleeding (muscles / haemarthroses) to mild

  • onset in early childhood

  • increased risk of post-operative / traumatic haemorrhage

Laboratory features

  • prolonged APTT

  • normal PT

  • normal bleeding time

  • plasma factor VIII reduced, von Willebrand factor normal

Treatment

  • registration with haemophilia centre + card with details of condition

  • avoid

    • aspirin,

    • antiplatelet drugs,

    • intramuscular injections

  • factor VII concentrate

    • plasma

    • recombinant

  • desmopressin

    • increases endogenous factor VIII

    • useful in mild cases

Complications

  • chronic joint disease

  • treatment using blood products has led to HIV and hepatitis C infection

  • neutralising antibodies to factor VIII

    • 15% of severely affected patients

    • immunosuppressive therapy

    • porcine factor VIII

    • plasma exchange

 

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