Surgery with emicizumab prophylaxis for two paediatric patients with severe haemophilia A with inhibitors

Emicizumab is a prophylaxis for patients with severe haemophilia A with and without inhibitor. Despite weekly administration of emicizumab, coagulation states stay below normal value and cannot be assessed by standard haemostasis tests. In our two patients, we used the thrombin-generation assay (endogenous thrombin potential and Peak) to monitor the patient's clotting status. Under emicizumab, it is necessary to add a bypassing agent (BPA) such as rFVIIa (Novoseven) to avoid bleeding before surgery. The BPA dosage was based on a thrombin-generation assay and collegial consultation.

Automated summary

For patients with severe hemophilia A receiving emicizumab treatment, it is important to adjust the dosage of bypassing agents based on thrombin-generation assay and collegial consultation to prevent bleeding risk before surgery.