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

Omri Cohen; Sarina Levy-Mendelovich; Ivan Budnik; Noa Ludan; Shani Kassia Lyskov; Tami Livnat; Einat Avishai; Orly Efros; Aharon Lubetsky; Shadan Lalezari; Mudi Misgav; Tami Brutman-Barazani; Gili Kenet; Assaf A Barg

doi:10.1016/j.rpth.2023.102178

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

Res Pract Thromb Haemost. 2023 Aug 23;7(6):102178. doi: 10.1016/j.rpth.2023.102178. eCollection 2023 Aug.

Authors

Omri Cohen^{1

2

3}, Sarina Levy-Mendelovich^{1

2

4}, Ivan Budnik⁵, Noa Ludan², Shani Kassia Lyskov², Tami Livnat^{1

2}, Einat Avishai^{1

2}, Orly Efros^{1

2}, Aharon Lubetsky^{1

2}, Shadan Lalezari^{1

2}, Mudi Misgav^{1

2}, Tami Brutman-Barazani^{1

2}, Gili Kenet^{1

2}, Assaf A Barg^{1

2}

Affiliations

¹ National Hemophilia Center, Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel-HaShomer, Israel.
² Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
³ Department of Medicine and Surgery, University of Insubria, Varese, Italy.
⁴ Sheba Talpiot Medical leadership program, Sheba Medical Center, Tel Hashomer, Israel.
⁵ Division of Hematology/Oncology, Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA.

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.