Severe protein C deficiency — defined by protein C activity below 1 IU/dL — substantially elevates thrombotic risk during the perioperative period. When an invasive surgical procedure cannot be deferred, specific perioperative management is required to support protein C activity throughout the operative window.
This protocol addresses patients with severe protein C deficiency — protein C activity confirmed at less than 1 IU/dL — who require an invasive surgical procedure. At this degree of deficiency, the coagulation risk is not adequately managed by standard perioperative measures alone; targeted protein C support is required to enable safe surgery.
Published experience confirms that major surgery has been successfully supported in patients with severe or moderately severe genetic protein C deficiency using protein C replacement as the perioperative intervention.
The protocol centres on protein C concentrate, given perioperatively to cover the surgical period. It specifies an initial bolus dose followed by subsequent maintenance dosing timed around the procedure — the precise quantities, dosing intervals, and infusion schedule are detailed in the full structured protocol.
The protocol aims to sustain a defined trough level of protein C activity across the perioperative period, together with a negative or rapidly decreasing D-dimer signal in the postoperative phase. Published data indicate that maintaining protein C concentrations within the therapeutic range around surgery has enabled invasive procedures to be performed with low risk of major haemorrhage, without requiring systemic anticoagulation.