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Poster Networking

Inherited Bleeding Disorders and Inhibitors

PB1252 - Surgical outcome of circumcision in a patient with hemophilia A with inhibitors on Emicizumab prophylaxis. Report of a case.

Tuesday, June 25, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall
Background: Circumcision is a widely used surgical procedure. In cases of hemophilia, circumcision is associated with several complications, including hemorrhage, infection, delayed healing, gangrene, risk of inhibitor development, psychosocial trauma, and risk of mortality.
Surgery is a challenge in patients with hemophilia A with inhibitors, in whom clotting factor replacement therapy is ineffective. A novel treatment in the prevention of bleeding includes Emicizumab, which is a bispecific monoclonal antibody that bridges activated FIXa and FX, substituting the function of missing FVIIIa, with resultant thrombin generation and fibrin clot formation.
According to the HAVEN 1-7 clinical trials, the efficacy and safety of Emicizumab were demonstrated in hemophilia A patients with or without FVIII inhibitors. However, there is limited real-world data.

Aims: To present the clinical case of a pediatric patient with severe hemophilia A on Emicizumab prophylaxis who underwent minor surgery.

Methods: Retrospective clinical case review.

Results: A 17-year-old male patient with diagnosis of severe hemophilia A with inhibitors (9.8 BU). He started Emicizumab with a loading dose weekly and later a 6mg/kg dose every 4 weeks. The patient presented with phimosis for which elective surgery (laser circumcision), was performed. Prior to surgery, inhibitor determination was 0.64 BU. In preparation for the procedure, he received a single dose of tranexamic acid 15mg/kg/do, minimal bleeding was reported and there was no need for to apply clotting factor concentrates, bypass agents, transfusion of hemocomponents, or other intervention. After two days with adequate evolution he was discharged with antibiotic and analgesic treatment with no further complications.

Conclusion(s): This is a case report real-experience which an antifibrinolytic was administered prior to surgery, without the need for clotting factor concentrate or bypass agent. The minor surgeries can be performed safely in patients with hemophilia A who receive Emicizumab prophylaxis. However, surgical guidelines need to be established for this population.