Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature

Abstract

Introduction Treatment of patients with Haemophilia A has improved significantly in recent years since the advent of novel therapeutic agents such as emicizumab. The low annualised bleeding rates associated with emicizumab have liberated many patients from the need for central venous access devices (CVAD). Optimal peri‐operative management of CVAD removal is not currently known and there are no specific formal recommendations available. Aim We reviewed outcomes in a paediatric cohort in our centre undergoing CVAD removal without pre‐operative factor or bypassing agent and reviewed the literature regarding port removal in patients on Emicizumab. Methods Ten male patients with severe Haemophilia A underwent CVAD removal without planned administration of factor concentrate or bypassing agent. Patients were monitored in hospital for 24 h with routine laboratory testing pre‐ and post‐operatively. Results No significant bleeding episodes occurred in any patient, no patient required factor concentrate or bypassing agent and no patients were readmitted due to bleeding within 7 days of surgery. Conclusion We propose that, in the era of emicizumab, prophylactic factor administration pre‐operatively for elective CVAD removal is not required in the majority of cases.

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“…All patients were discharged the day after surgery and no patient required any additional haemostatic therapy except one, who received oral tranexamic acid for five postoperative days due to minor bleeding from the surgical site. 47 Similar outcomes have been reported for a total of additional 27 CVAD removals included in the description of other case series on emicizumab use. [66][67][68][69] Of these only five received preoperative treatment 66 and only one experienced a mild postoperative bleeding.…”

“…recently reported on 10 children (1 with current inhibitors) with severe HA who underwent port removal without any planned preprocedural prophylactic factor administration. All patients were discharged the day after surgery and no patient required any additional haemostatic therapy except one, who received oral tranexamic acid for five postoperative days due to minor bleeding from the surgical site 47 . Similar outcomes have been reported for a total of additional 27 CVAD removals included in the description of other case series on emicizumab use 66–69 .…”

“…32 Surgical experience in patients on emicizumab out of clinical trials is being increasingly reported in form of either case reports 34,[37][38][39]41,[43][44][45][46][64][65] or case series. 33,40,42,47 Table 2 summarizes the real-world experience reported so far in form of case reports.…”

“…To date, limited information on surgical management of PWH on prophylaxis with fitusiran and anti‐TFPI is available from clinical trials 27–29 . On the other hand, more data have been collected and published on the surgical management of patients on emicizumab prophylaxis both during the clinical trial program 30–32 and in the real‐world setting 33–47 …”

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“…The removal of central venous access devices was performed in ten male pediatric patients with severe form of hemophilia A who were on prophylactic treatment with emicizumab without administration of factor VIII concentrate or bypassing agent. There were no significant hemorrhages and no patient returned to the hospital in the week following the intervention 51 .…”