OC 14.3 - Management and outcomes of surgical procedures in people with haemophilia receiving emicizumab

Background: Emicizumab, a monoclonal antibody that mimics Factor VIII, has revolutionised the treatment of people with severe haemophilia A. The optimal management of these patients who undergo minor and major surgery remains undefined.

Aims: Review the management and outcomes of surgical procedures in people with haemophilia receiving emicizumab.

Methods: Retrospective cohort study of all patients receiving emicizumab who underwent a surgical procedure between November 2019 and December 2023. Severity of bleeding was defined as per ISTH classification.

Results: Of 98 people with severe haemophilia A in our centre receiving emicizumab, 27 underwent 36 procedures. Twenty-six were minor and 10 were major. Baseline characteristics are in Table 1. Of the 26 minor procedures, 4 proceeded with tranexamic acid alone while the remainder (23, 88%) received peri-operative recombinant factor VIII (rFVIII) concentrate (see table 2). One patient had a major bleed after a haemorrhoidectomy despite pre-operative rFVIII and required treatment with rFVIII for seven days. One patient had non-major bleeding after ligation of arteriovenous fistula, requiring three doses of rFVIII. All ten major surgeries were covered with factor concentrate pre- and postoperative. Median pre-operative dose was 3000 units (IQR 2000, 3875), 32 IU/kg (IQR 25, 44) and achieving a median peak FVIII level of 129 iu/dL. Two patients with inhibitors received recombinant factor VIIa (rFVIIa); the remaining seven received postoperative rFVIII at a median cumulative dose of 57500 units (706 IU/kg). One patient experienced intra-articular bleeding following ankle arthroscopy requiring prolonged use of factor concentrate (total dose of 157000 units). There was no other bleeding. There were no cases of venous or arterial thrombosis.

Conclusion(s): Surgery in patients receiving emicizumab together with rFVIIa or rFVIII coagulation factor replacement can be safely managed without an increased risk of thrombosis