Management of people with haemophilia A undergoing surgery while receiving emicizumab prophylaxis: Real‐world experience from a large comprehensive treatment centre in the US

Abstract

Introduction Surgery is frequently required in persons with haemophilia A (PwHA). Emicizumab, a bispecific, humanized monoclonal antibody, bridges activated factor (F) IX and FX. Management of patients undergoing surgery while receiving emicizumab is of clinical interest due to paucity of data. Aim Review real‐world experience of PwHA with/without FVIII inhibitors who required surgery while receiving emicizumab prophylaxis. Methods Data regarding peri‐operative management, including type of surgery, haemostatic agent use and bleeding complications, were collected for PwHA receiving emicizumab undergoing surgery between 25/10/18 and 31/12/19 at the Indiana Hemophilia and Thrombosis Center. Analyses were exploratory and descriptive. Results Twenty minor and five major surgeries were performed in 17 and five patients, respectively. Overall, 9/20 minor surgeries were planned to occur with emicizumab as the sole haemostatic agent; of these, four required additional coagulation factor (2 due to haematomas following port removals, 1 due to oozing at port removal site, 1 due to bleeding following squamous cell carcinoma removal). Three of the 11 minor surgeries with planned additional coagulation factor resulted in non‐major bleeds; all were safely managed with additional coagulation factor. All five major surgeries were planned with additional haemostatic agents; there was 1 bleed in a patient undergoing elbow synovectomy with nerve transposition, likely triggered by physical/occupational therapy. There were no major bleeds, thrombotic events or deaths. Conclusions Additional haemostatic agent use is safe in PwHA undergoing surgery while receiving emicizumab. Additional data are needed to determine the optimal dosing/length of treatment of additional haemostatic agents to lower bleeding risk.

Adoption of emicizumab (Hemlibra®) for hemophilia A in Europe: Data from the 2020 European Association for Haemophilia and Allied Disorders survey

“…Results of the EAHAD survey are mostly in line with these recommendations and with published data from the HAVEN studies and two HTCs in the US 15,16,17 . Regarding bypassing agents, rFVIIa is the treatment of choice for patients with inhibitors on emicizumab 10,11,13,14,16 . Survey results are consistent with this recommendation.…”

“…For example, data on management of inhibitor and non‐inhibitor patients on emicizumab undergoing surgery is currently scarce and no guidelines on a European or global level, only proposals and recommendations on a national level, are available so far 12,13,14 . Results of the EAHAD survey are mostly in line with these recommendations and with published data from the HAVEN studies and two HTCs in the US 15,16,17 . Regarding bypassing agents, rFVIIa is the treatment of choice for patients with inhibitors on emicizumab 10,11,13,14,16 .…”

Management and outcomes of paediatric patients on emicizumab prophylaxis undergoing surgical procedures: Experience from a large haemophilia centre in the UK

“…During these trials 141/215 (65.6%) of minor surgeries/procedures, including dental and central venous access device removals, were managed without prophylactic coagulation factor, and 128/141 (90.8%) did not result in post-operative bleeding requiring factor replacement. 10 Similarly, Lewandowska et al 3 reported that five out of nine (5/9) vascuport removals were successfully performed with emicizumab as a sole haemostatic agent. Moreover, in an observational study from three haemophilia centres in the United States, one major and 28 minor surgical procedures were performed in PwHA being treated with emicizumab prophylaxis.…”

“…In the emicizumab era, CVAD devices removal is a common procedure as venous infusions are no longer needed and are replaced by subcutaneous injections. 3 However, there is no consensus on the appropriate management of CVAD removals or other surgical procedures in patients on this novel agent. 4 The safety and efficacy of emicizumab prophylaxis in PwHA have been demonstrated in HAVEN clinical trials programme.…”

“…[5][6][7][8] Although minor and unplanned major surgical procedures did occur during HAVEN trials, patients were managed according the investigator's decision. 3 This letter presents real world experience of management and outcomes of paediatric PwHA with and without inhibitors who underwent surgical procedures while on emicizumab prophylaxis in a large comprehensive haemophilia centre in the United Kingdom.…”