Patients with hemophilia A (HA) frequently develop neutralizing antibodies (inhibitors) against exogenous coagulation factor Ⅷ (FⅧ) due to repeated infusion. Perioperative hemostatic management presents a considerable clinical challenge in high-responder inhibitor patients experiencing severe trauma or major orthopedic surgery. This report describes the case of a 22-year-old patient with severe HA and high-titer FⅧ inhibitors, who sustained a distal femoral fracture complicated by osteofascial compartment syndrome and neurovascular injury. Recombinant activated factor Ⅶ (rFⅦa) was initially selected as the first-line bypassing agent during the perioperative period; however, due to economic constraints, a combined regimen of rFⅦa and prothrombin complex concentrate (PCC) was ultimately employed alongside FⅧ infusion to achieve comprehensive hemostasis, thereby facilitating successful open reduction and internal fixation. In conjunction with a review of the literature, this article focuses on the dosing strategy of rFⅦa as the primary perioperative intervention, real-world challenges, and the implementation of integrated hemostatic protocols in HA patients with high-titer inhibitors.

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