Hemophilia A&B 

 

 

Background 

 

  • X-linked recessive disorder characterized by a deficiency of Factor 8 (A) or 9 (B) resulting in spontaneous hemorrhage or uncontrolled bleeding with trauma or surgery

  • Classification by factor levels:

    • Mild:  5-25% 

    • Moderate: 1-5%  

    • Severe: <1%  

  • ↑ PTT, normal INR 

 

 

Considerations 

 

  • High risk for perioperative bleeding

  • Sequelae of bleeding into enclosed spaces (joints, intracranium, pericardium, thorax)

  • Potential contraindication to neuraxial anesthesia 

  • Factor optimization (replacement/supplementation) & identification of factor antibodies (e.g., inhibitors)

  • Coexisting viral infections secondary to transfusions: HIV, hepatitis (less now with recombinant products)

  • Consider preoperative hematology consultation

 

 

Goals

 

  • Optimize factor activity & coagulation profile in perioperative period

  • Minimize perioperative blood loss; consider blood conservation strategies

 

 

Optimization & Treatment 

 

  • Hemophilia A

    • DDAVP for mild disease 

    • Recombinant factor VIII (Humate P) 

    • Factor VIII concentrates (fresh frozen plasma contains minimal factor VIII)

    • Recombinant factor VIIa for inhibitors (approved indication)

    • Cryoprecipitate if nothing else available (this is the only standard fractionated blood product containing meaningful amounts of factor VIII)

      • Cryoprecipitate contains factor VIII, fibrinogen, von Willebrand factor, fibronectin, factor XIII

  • Hemophilia B

    • Recombinant factor IX

    • Factor IX concentrates

 

 

Treatment Factor Levels 

 

 

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