Abstract Number: PB1154
Meeting: ISTH 2020 Congress
Background: Emicizumab is a humanised bi-specific monoclonal antibody functionally similar to factor VIII (FVIII) with demonstrated efficacy of haemostatic prophylaxis in boys with severe haemophilia A (SHA) and FVIII inhibitor. There is limited surgical data in patients on emicizumab and the treating-community are still establishing best practice guidance.
Aims: To describe single-centre experience with central venous access device (CVAD) removal in boys with SHA and inhibitors on treatment with emicizumab.
Methods: Twenty-three boys are currently on treatment with emicizumab at Great Ormond Street Hospital. We present a case series of ten boys who had CVAD removal while on prophylaxis with emicizumab.
Results: Nine boys who underwent surgical CVAD removal were on weekly emicizumab dosing and one on monthly dosing. All had received emicizumab and tranexamic acid (TXA) the day before surgery. The median age at procedure was 7years (range 2- 14years) and median time on emicizumab at the time of procedure was 39 weeks (range 8- 80 weeks). There was no recorded blood loss during any procedure and all patients were discharged the following day with a plan to continue TXA (15mg/kg/dose three-times daily) for 10 days. None of the patients required rescue by-passing agent treatment for bleeding. One patient was readmitted within 24 hours with swelling of the CVAD site, an ultrasound of the CVAD area revealed a haematoma (8cms diameter) which responded rapidly to re-starting tranexamic acid. This patient had erroneously stopped TXA after discharge.
Conclusions: Surgical removal of CVAD was carried out with TXA cover only in 10 patients. One had a minor bleeding complication due to early cessation of TXA which responded to re-starting the medication. This cohort demonstrates that TXA given regularly at doses of 15mg/kg three-times daily to start before surgery and continued for 10 days afterwards is successful in securing haemostasis during CVAD removal in children on emicizumab.
To cite this abstract in AMA style:Badle S, Gosrani D, Taylor A, Sibson K, Mathias M, Liesner R. Surgical Experience with Emicizumab: A Single Centre Experience with Central Venous Access Device Removal [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/surgical-experience-with-emicizumab-a-single-centre-experience-with-central-venous-access-device-removal/. Accessed February 26, 2024.
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