Short and Long-Term Outcomes in Hemophilia Patients with Inhibitors Undergoing Orthopedic Prosthetic Surgery

Introduction: Major orthopedic surgery is still a difficult challenge in hemophilic patients with high titer of inhibitor. In addition to possible peri-operative problems, the long-term outcomes are anecdotal.

Aim of the study: to evaluate the short and long term outcomes in prosthetic orthopedic procedures done in hemophilic patients with inhibitor from 1998 to 2014

Results: Sixteen knee replacements have been performed in 12 patients affected by hemophilia A with inhibitors. At the time of surgery 11 out of 12 patients had an inhibitor titer > 5UB (range 5 - 2,500). A preoperative pharmacokinetic with rFVIIa was made in 8 out of 12 patients, with the following mean values of clearance rate: 35 ml/h/kg and half-life: 2.85 hours. The average age was 46 years (range 29-55). All patients were treated with 2-3 doses of rFVIIa (90-120 mg/kg) every two hours during surgery up to suturing of the wound, followed by continuous infusion of rFVIIa at dose of 30-50 mg/kg/h on days 1-3 and 15 mg/kg/h on days 4-14. Plasma levels of FVII:C were maintained > 15U/mL. All but one patients were transfused with 2 units of red blood cells (RBC), according to standard procedure. Two peri-operative complications occurred: 1 pulmonary embolism and 1 major bleeding at the operated site, poorly responsive to rFVIIa and aPCC, treated with 6 units of RBC. Only two patients underwent to prosthesis revision for infection, while another due to rupture of the prosthesis metal cup. After surgery all patients showed a significant improvement in pain and a favorable functional recovery. After a median follow-up of 7 years (1-18 years), a good quality of life as to pain and function of the replaced knee were recorded.

Conclusions: The orthopedic prosthetic surgery in hemophilic patients with inhibitors can be done, with a good outcome. The continuous infusion of rFVIIa provides stable haemostatic coverage, saving on the rFVIIa total dose compared to the bolus standard dose. However, costs remain high and the possible risks of thromboembolic complications should not be overlooked