Management of a High-Risk Surgery with Emicizumab and Factor VIII in a Child with a Severe Hemophilia A and Inhibitor

Authors : Lefèvre CR.; Jaffré, A; Pontis, A; Nedelec-Gac, F; Guéret, P; Gouin-Thibault, I; Fraisse, B; Bayart, S; Guillet, B.

Affiliations: Laboratoire d’Hémostase Bioclinique, CHU de Rennes, Bretagne, France ; Centre Régional de Traitement de l’Hémophilie, CHU de Rennes, Bretagne, France; Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) – UMR_S 1085, Rennes, France ; Service de Réanimation Pédiatrique, CHU de Rennes, Bretagne, France ; Laboratoire d’Hémostase Bioclinique, CHU de Rennes, Bretagne, France ; Service de Chirurgie Pédiatrique, CHU de Rennes, Bretagne, France.

Publication : Thrombosis and Haemostasis open; 2021 ; 5. e163-e165

Abstract: The recent development of a humanized, bi-specific, and monoclonal antibody mimicking the function of activated factor VIII was a revolution in the management of patients suffering from severe hemophilia A with inhibitors.[1] The phase III randomized studies have shown a more efficient prophylaxis of this subcutaneous administered drug in these patients compared with recombinant FVIIa (rFVIIa) and activated prothrombin complex concentrates (aPCC).[2] [3] Nonetheless, there are “real life” matters that need to be explored in this new era of managing hemophilia patients, such as surgery management under emicizumab, especially in children.[4] [5] [6] Here, we report the first case, to our knowledge, of major orthopedic surgery managed with factor VIII infusions in a child with inhibitor receiving emicizumab.