From the Guidelines
Preoperative management of Factor XI deficiency, also known as Hemophilia C, should involve the administration of plasma-derived Factor X (pdFX) concentrate to raise plasma FX:C levels to 70-90 IU/dL 1-4 hours before surgery, as this approach has been shown to be effective and safe in achieving excellent hemostatic efficacy. The management of patients with Factor XI deficiency undergoing surgery has evolved over time, with earlier cases managed using fresh frozen plasma (FFP), prothrombin complex concentrates (PCCs), and factor IX/X concentrate 1. However, with the availability of pdFX concentrate, this has become the preferred treatment modality. According to a prospective, open-label phase 3 study, pdFX infusion was effective in achieving excellent hemostatic efficacy in patients with severe, moderate, and mild Factor XI deficiency undergoing major and minor surgical procedures 1. The study demonstrated that pdFX infusion could maintain FX:C levels above 50 IU/dL until the patient was no longer considered to be at risk of postoperative bleeding, with no adverse drug reactions, hypersensitivity reactions, thrombotic events, or evidence of inhibitor development 1. Key considerations in the preoperative management of Factor XI deficiency include:
- Individualized assessment based on the patient's baseline Factor XI level, bleeding history, and surgical bleeding risk
- Administration of pdFX concentrate to achieve target FX:C levels
- Monitoring of FX:C levels and adjustment of pdFX dosage as needed
- Consideration of adjunctive therapies, such as tranexamic acid, especially for mucosal surgeries
- Close monitoring for bleeding and continuation of hemostatic therapy until wound healing is adequate.
From the Research
Preoperative Management for Factor XI Deficiency
The preoperative management for Factor XI deficiency (Hemophilia C) involves several strategies to minimize the risk of bleeding during surgery. Some of the approaches include:
- Administering fresh frozen plasma (FFP) or thawed plasma to increase factor XI levels 2, 3, 4
- Using therapeutic plasma exchange (TPE) as an alternative perioperative management strategy to reduce the risk of volume overload 2
- Infusing factor XI concentrates preoperatively, if available 5
- Administering tranexamic acid to reduce fibrinolysis 3, 5
- Transfusing platelets and fibrinogen during surgery, if necessary 5
- Closely monitoring perioperative factor levels and activated partial thromboplastin time (aPTT) values 3
Treatment Options
Treatment options for factor XI-deficient patients include:
- Fresh frozen plasma (FFP) 2, 3, 4
- Factor XI concentrates (available in some countries) 6, 5
- Antifibrinolytics, such as tranexamic acid 3, 5
- Recombinant factor VIIa 6
- 4-factor prothrombin complex concentrate (4-factor PCC) 6
Perioperative Management
Perioperative management of factor XI deficiency involves: