Hemophilia C
ICD-10 D68.1 · ICD-11 3B13

Treatment of Hemophilia C with Severe Factor XI Deficiency Undergoing High-Bleeding-Risk Surgery

Patients with Hemophilia C who have severe factor XI deficiency — an FXI:C level below approximately 20 U/dL — face substantial haemorrhagic risk during procedures such as tonsillectomy or prostatectomy. This clinical scenario requires structured perioperative haemostatic management.

Clinical Scenario

Severe factor XI deficiency significantly elevates bleeding risk at surgery, particularly for procedures known to carry high haemorrhagic potential. Most patients with this degree of deficiency require active haemostatic support around the time of surgery. Patient age and individual comorbidity profile both influence the selection and conduct of treatment.

Treatment Approach (Overview)

The perioperative approach centres on factor XI replacement by infusion, with important restrictions regarding the concurrent use of certain other haemostatic agents.

Complete dosing criteria, product selection, and the full management algorithm are available in the structured protocol below.

Clinical Goal

The primary treatment target is a post-infusion factor XI activity level (FXI:C) not exceeding 100 U/dL — achieving adequate haemostasis without over-correction.

Instant Access to Structured Evidence-Based Regimens

References

Factor XI concentrate may be indicated for procedures with a significant risk of bleeding, especially in younger patients with severe deficiency, but its use in older patients has been associated with thrombotic phenomena.
Most people with severe deficiency are at risk of hemorrhage after procedures such as tonsillectomy or prostatectomy and will benefit from concentrate therapy.
Concurrent use of tranexamic acid or other antifibrinolytic drugs should be avoided.
UKHCDO Haemophilia Centre Doctors' Organisation guidelines recommend that dosage does not exceed 30 U/kg and that the post infusion FXI:C level should not exceed 100 U/dL.
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