Hemophilia A&B
Background
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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
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Classification by factor levels:
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Mild: 5-25%
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Moderate: 1-5%
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Severe: <1%
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↑ PTT, normal INR
Considerations
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High risk for perioperative bleeding
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Sequelae of bleeding into enclosed spaces (joints, intracranium, pericardium, thorax)
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Potential contraindication to neuraxial anesthesia
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Factor optimization (replacement/supplementation) & identification of factor antibodies (e.g., inhibitors)
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Coexisting viral infections secondary to transfusions: HIV, hepatitis (less now with recombinant products)
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Consider preoperative hematology consultation
Goals
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Optimize factor activity & coagulation profile in perioperative period
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Minimize perioperative blood loss; consider blood conservation strategies
Optimization & Treatment
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Hemophilia A
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DDAVP for mild disease
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Recombinant factor VIII (Humate P)
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Factor VIII concentrates (fresh frozen plasma contains minimal factor VIII)
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Recombinant factor VIIa for inhibitors (approved indication)
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Cryoprecipitate if nothing else available (this is the only standard fractionated blood product containing meaningful amounts of factor VIII)
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Cryoprecipitate contains factor VIII, fibrinogen, von Willebrand factor, fibronectin, factor XIII
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Hemophilia B
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Recombinant factor IX
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Factor IX concentrates
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Treatment Factor Levels
