Abstract Number: PB0977
Meeting: ISTH 2020 Congress
Background: Surgical procedures in Haemophilia A with inhibitors is challenging with varrying bleeding patterns. Though emicizumab concurs almost normal haemostasis in most patients, there is little experience on it´s efficacy in surgical procedures.
Aims: Report our experience in managing Haemophilia A with inhibitor on emicizumab.
Methods: Case report.
Results: This 17 year old was diagnosed with Severe Haemophilia A at 12 months and developed high titre inhibitor within the first 50 exposure days. Immune tolerance induction (ITI) was attempted at 10 years but failed. He was on-demand therapy with an annualised bleeding rate of 100 per year.
In March 2019, he fortunately received Emicizumab on compassionate use program.
He received loading dose of 150mg weekly (2mg/kg) for 4 weeks followed by 150mg once every 2 weeks . This dose was chosen as only 150 mg vials were approved for compassionate use and the decision was to under dose him to prevent wastage.
Despite this reduced dose, he showed significant improvement in bleeding pattern. He stopped having weekly bleeds, was able to go to school daily and also managed some light sports.
In January 2020, during dental follow up it was noted that his lower 3rd molar has carries requiring extraction. He injected his routine emicizumab dose 3 days before planned extraction and took oral transxenamic acid 1g TDS starting the day before procedure. Simple extraction was done and suture applied to secure haemostasis. No excessive bleeding was seen immediately post extration but 4 hours later there was a small haematoma. This resolved with transxenamic acid and ice gurgle. There was no further bleeding and no bypassing agents required. He continues with transxenamic acid and is due for suture removal in a couple of days.
Conclusions: Haemophilia A with inhibitors on emicizumab can possibly be managed without bypassing agents or blood test for simple surgical procedures.
To cite this abstract in AMA style:Selvaratnam V, Rui Jeat F, Yee Yee Y, Sathar J. Emicizumab and Surgery in Severe Haemophilia A with Inhibitor: Our First Experience [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/emicizumab-and-surgery-in-severe-haemophilia-a-with-inhibitor-our-first-experience/. Accessed February 28, 2024.
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