Management of surgery in persons with hemophilia A receiving emicizumab prophylaxis: data from a national hemophilia treatment center

Abstract

Background: Persons with hemophilia A may require surgical procedures. Real-world data on invasive procedures in persons with hemophilia A receiving emicizumab prophylaxis are limited.

Objectives: To evaluate the safety of invasive procedures in persons with hemophilia A receiving emicizumab prophylaxis and their outcomes in a longitudinally followed cohort.

Methods: Data from medical records of persons with hemophilia A with and without factor VIII (FVIII) inhibitors longitudinally followed at our tertiary center, who received emicizumab prophylaxis and underwent all types of invasive procedures, were retrieved. Outcomes of interest were bleeding and thrombotic complications.

Results: Overall, 35 patients underwent 56 invasive procedures, 18 (32.1%) were major. The median age was 36.3 years (IQR, 8.8-55.9 years); 12 patients (34.3%) were younger than 18 years at the time of procedure; 17 (48.6%) were patients with FVIII inhibitors. Among major procedures, orthopedic surgeries prevailed. All patients who underwent major procedures received factor replacement with either recombinant activated factor VII (patients with inhibitors) or FVIII (patients without inhibitors). Factor concentrates were administered prior to 32 (84.2%) of the minor procedures. Repeated doses were given according to international expert opinion recommendations and patients' condition.There were 7 bleeding events in 6 patients, 5 were major bleeds, including 1 patient who underwent a minor procedure without factor replacement. None of the patients experienced a thrombotic complication.

Conclusion: Invasive procedures can be performed safely in patients receiving emicizumab prophylaxis with close surveillance after surgery. Factor concentrates may be advised in selected patients undergoing minor procedures.

Keywords: emicizumab; hemophilia A; inhibitors; invasive procedures; surgery.

Figure

Hemostatic impact of recombinant activated factor FVII (rFVIIa) concentrate on thrombin generation (TG) peak height and endogenous thrombin potential in plasma samples of persons with severe hemophilia A receiving once weekly emicizumab prophylaxis. (A) The hemostatic impact of rFVIIa concentrate on TG peak height and endogenous thrombin potential in a plasma sample of a person with severe hemophilia A and a high responding inhibitor receiving once weekly emicizumab prophylaxis, in the presence of 1 pM tissue factor, as compared with a control (healthy subject). (B) TG of plasma obtained from an emicizumab-treated patient with a high responding inhibitor, before and after surgery, 30 minutes after perioperative administration of 66 μg/kg of rFVIIa concentrate.