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Surgery with emicizumab prophylaxis for two paediatric patients with severe haemophilia A with inhibitors
Author(s) -
Lockhart Marguerite,
TardyPoncet Brigitte,
Montmartin Aurélie,
Noyel Pauline,
Thouvenin Sandrine,
Berger Claire
Publication year - 2021
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.29041
Subject(s) - medicine , haemophilia , coagulation , haemophilia a , thrombin generation , thrombin , surgery , platelet
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.