Perioperative management of patients with heterozygous protein C deficiency is challenging because of the competing risks of bleeding and recurrent thrombosis.
We report the case of a 74-year-old man with protein C deficiency and heterozygous prothrombin G20210A gene mutation who had a successful left THA with perioperative administration of human zymogen protein C concentrate in addition to anticoagulation with enoxaparin.
Several studies have reported the use of protein C concentrate in severe sepsis-associated purpura fulminans in patients with severe congenital protein C deficiency who have had thrombotic events. We reviewed studies and case reports pertinent to the treatment of patients with protein C deficiency, especially in the perioperative setting. We report the case of a patient undergoing THA in whom we used human zymogen protein C concentrate.
THA, a particularly high-risk procedure, is associated with a 40% to 70% incidence of venographic deep venous thrombosis and a 2% to 3% incidence of symptomatic deep venous thrombosis. These risks are greater in people with thrombophilic defects such as protein C deficiency. The use of human zymogen protein C in our patient with heterozygous protein C deficiency during the perioperative period of a THA was associated with no evidence of excessive bleeding, hematoma, deep venous thrombosis, or pulmonary embolism.