Surgery with emicizumab prophylaxis for two pediatric patients with
severe haemophilia A with inhibitors
Abstract
Emicizumab is a prophylaxis for patients with severe haemophilia A with
inhibitor. Despite the daily prophylaxis, coagulation states stay below
normal value and cannot be assessed by standard hemostasis techniques.
In our two patients, we use thrombin generation assay (endogenous
thrombin potential (ETP) and Peak) to monitor the patient’s clotting
status. Under emicizumab, it is needed to add by-passing agent such as
FVIIa (Novoseven®) to avoid bleeding before surgery. By-passing agent
dose was chosen with the help of thrombin generation assay and after
collegial concertation.