PB0627 - Management of invasive procedures in patients with Hemophilia A receiving Emicizumab prophylaxis: Real-world data from a national Hemophilia treatment center.

Monday, June 26, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Omri Cohen, MD

Senior physician
Sheba medical center, Tel Aviv University
Holon, HaMerkaz, Israel

Co-Author(s)

IB

Ivan Budnik

MD Phd
University of IOWA
Iowa city, Iowa, United States
NL

Noa Ludan

Student
Sackler School of Medicine, Tel Aviv University, Israel
Tel Aviv, Tel Aviv, Israel
SK

Shani Kassia Lyskov

Student
Sackler School of Medicine, Tel Aviv University, Israel
Tel Aviv, Tel Aviv, Israel
Orly Efros, MD, MHA (she/her/hers)

Physician, thrombosis and hemostasis specialist
National Hemophilia Center and Thrombosis & Hemostasis Institute, Sheba Medical Center, Israel. Sackler Faculty of Medicine, Tel Aviv University, Israel.
Rishon Letzion, HaMerkaz, Israel
AL

Aharon Lubetsky

Director, Thrombosis and Hemostasis Unit
National Hemophilia Center and Thrombosis & Hemostasis Institute, Sheba Medical Center, Israel. Sackler Faculty of Medicine, Tel Aviv University, Israel.
Ramat Gan, HaMerkaz, Israel
SL

Shadan Lalezari

Senior physician
Sheba medical center, Tel Aviv University
Netanya, HaMerkaz, Israel
MM

Mudi Misgav

Head of blood bank
Sheba medical center, Tel Aviv University
Tel Aviv, Tel Aviv, Israel
Gili Kenet, MD

Director
The National Hemophilia Center, and Amalia Biron Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Tel Aviv University, Tel Aviv, Israel
Yehud-Monosson, HaMerkaz, Israel
Assaf Barg, MD

Pediatric Hematologist
The National Hemophilia Center, and Amalia Biron Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Ramat Gan, Israel
Tel Aviv, HaMerkaz, Israel

Background: Emicizumab prophylaxis supports hemostasis in patients with Hemophilia A (PwHA) with and without inhibitors to Factor VIII (FVIII). Data from the HAVEN studies previously demonstrated that PwHA receiving emicizumab prophylaxis can safely undergo minor and major surgeries, although major surgeries often require additional FVIII or recombinant Factor VIIa (rFVIIa) prophylaxis. Real world data on invasive procedures in PwHA receiving emicizumab prophylaxis are limited.

Aims: To evaluate the safety of invasive procedures in PwHA receiving emicizumab prophylaxis and their outcomes in a longitudinally followed real world cohort.

Methods: Prospectively collected data from medical records of PwHA with and without FVIII inhibitors receiving emicizumab prophylaxis, who underwent all types of invasive procedures were retrieved. Outcomes of interest were bleeding of any severity and thrombotic complications. The study was approved by an institutional review board, and all patients provided informed consent.

Results: Overall, 35 patients underwent 56 invasive procedures, 18 (32.1%) were major. The median (interquartile range [IQR]) age was 37 (IQR 10-56) years, 12 patients (34.3%) were younger than 18 years at the time of procedure, 26 (74.3%) were patients with FVIII inhibitors. Among major procedures, orthopedic surgeries prevailed (Table 1). All patients who underwent major procedures received factor replacement either with rFVIIa 90 μg/kg (patients with inhibitors, n=15) or FVIII 35-50 IU/kg (patients without inhibitors, n=3). Factor concentrates were administered prior to 32 (84%.2) of minor procedures. Repeated doses were given according to international expert opinion recommendations and patients’ condition.
Six patients experienced bleeding, 4 were major bleeds, including one patient who underwent a minor procedure without factor replacement (Table 2). None of the patients experienced a thrombotic complication.

Conclusion(s): Invasive procedures can be performed safely in patients receiving emicizumab prophylaxis. Factor concentrates may be advised in selected patients undergoing minor procedures.