Challenges of biological monitoring in a hemophilia A patient without inhibitors on emicizumab undergoing major orthopedic surgery: a case report

1. Srivastava, A, Santagostino, E, Dougall, A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia 2020; 26: 1–158. DOI: 10.1111/hae.14046.
Google Scholar | Crossref | Medline2. Mahlangu, J, Oldenburg, J, Paz-Priel, I, et al. Emicizumab prophylaxis in patients who have hemophilia A without inhibitors. N Engl J Med 2018; 379: 811–822. DOI: 10.1056/nejmoa1803550.
Google Scholar | Crossref | Medline3. Seaman, CD, Ragni, MV. Emicizumab use in major orthopedic surgery. Blood Adv 2019; 3: 1722–1724. DOI: 10.1182/bloodadvances.2019000228.
Google Scholar | Crossref | Medline4. Nougier, C, Jeanpierre, E, Ternisien, C, et al. Emicizumab treatment: impact on coagulation tests and biological monitoring of haemostasis according to clinical situations (BIMHO group proposals). Eur J Haematol 2020; 105: 675–681. DOI: 10.1111/ejh.13490.
Google Scholar | Crossref | Medline5. Jenkins, PV, Bowyer, A, Burgess, C, et al. Laboratory coagulation tests and emicizumab treatment A United Kingdom Haemophilia Centre Doctors’ Organisation guideline. Haemophilia 2020; 26: 151–155. DOI: 10.1111/hae.13903.
Google Scholar | Crossref | Medline6. Pipe, SW, Shima, M, Lehle, M, et al. Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study. Lancet Haematol 2019; 6: e295–e305. DOI: 10.1016/S2352-3026(19)30054-7.
Google Scholar | Crossref | Medline7. https://ondpanon.riziv.fgov.be/SSPWebApplicationPublic/fr/Public/ProductSearch (accessed 12 November 2020).
Google Scholar8. Prelog, T, Dolnicˇar, MB, Kitanovski, L. Low-dose continuous infusion of factor VIII in patients with haemophilia A. Blood Transfus 2016; 14: 474–480. DOI: 10.2450/2015.0080-15.
Google Scholar | Crossref | Medline9. Park, YS, Shin, WJ, Kim, K, et al. Comparison of continuous infusion versus bolus injection of factor concentrates for blood management after total knee arthroplasty in patients with hemophilia. BMC Musculoskelet Disord 2017; 18: 1–5. DOI: 10.1186/s12891-017-1720-0.
Google Scholar | Crossref | Medline10. Susen, S, Gruel, Y, Godier, A, et al. Management of bleeding and invasive procedures in haemophilia A patients with inhibitor treated with emicizumab (Hemlibra®): proposals from the French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia, in collaboration with the French Working Group on Perioperative Haemostasis (GIHP). Haemophilia 2019; 25: 731–737. DOI: 10.1111/hae.13817.
Google Scholar | Crossref | Medline11. Coppola, A, Castaman, G, Santoro, RC, et al. Management of patients with severe haemophilia a without inhibitors on prophylaxis with emicizumab: AICE recommendations with focus on emergency in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET. Haemophilia 2020; 26: 937–945. DOI: 10.1111/hae.14172.
Google Scholar | Crossref | Medline12. Lowe, A, Kitchen, S, Jennings, I, et al. Effects of Emicizumab on APTT, FVIII assays and FVIII Inhibitor assays using different reagents: results of a UK NEQAS proficiency testing exercise. Haemophilia 2020; 26: 1087–1091. DOI: 10.1111/hae.14177.
Google Scholar | Crossref | Medline13. Müller, J, Pekrul, I, Pötzsch, B, et al. Laboratory monitoring in emicizumab-treated persons with hemophilia A. Thromb Haemost 2019; 119: 1384–1393. DOI: 10.1055/s-0039-1692427.
Google Scholar | Crossref | Medline14. Fontana, P, Alberio, L, Albisetti, M, et al. Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab. Swiss Med Wkly 2020; 150: w20422. DOI: 10.4414/smw.2020.20422.
Google Scholar | Crossref15. Ferrière, S, Peyron, I, Christophe, OD, et al. A hemophilia A mouse model for the in vivo assessment of emicizumab function. Blood 2020; 136: 740–748. DOI: 10.1182/blood.2019004334.
Google Scholar | Crossref | Medline16. Bravo, MI, Raventós, A, Pérez, A, et al. Non-additive effect on thrombin generation when a plasma-derived factor VIII/von Willebrand factor (FVIII/VWF) is combined with emicizumab in vitro. J Thromb Haemost 2020; 18: 1934–1939. DOI: 10.1111/jth.14887.
Google Scholar | Crossref | Medline17. Makris, M, Iorio, A, Lenting, PJ. Emicizumab and thrombosis: the story so far. J Thromb Haemost 2019; 17: 1269–1272. DOI: 10.1111/jth.14556.
Google Scholar | Crossref | Medline18. Nakajima, Y, Nogami, K, Yada, K, et al. Emicizumab improves ex vivo clotting function in patients with mild/moderate hemophilia A. Thromb Haemost 2020; 120: 968–976. DOI: 10.1055/s-0040-1710315.
Google Scholar | Crossref | Medline19. Santagostino, E, Mancuso, ME, Novembrino, C, et al. Rescue factor VIII replacement to secure hemostasis in a patient with hemophilia A and inhibitors on emicizumab prophylaxis undergoing hip replacement. Haematologica 2019; 104: e380–e382. DOI: 10.3324/haematol.2018.215129.
Google Scholar | Crossref | Medline20. Biron-Andreani, C, Diaz-Cau, I, Ranc, A, et al. Major surgery management in patients with haemophilia A and inhibitors on emicizumab prophylaxis without global coagulation monitoring. Br J Haematol 2020; 189: e100–e103. DOI: 10.1111/bjh.16512.
Google Scholar | Crossref | Medline21. Kizilocak, H, Yukhtman, CL, Marquez-Casas, E, et al. Management of perioperative hemostasis in a severe hemophilia A patient with inhibitors on emicizumab using global hemostasis assays. Ther Adv Hematol. Epub ahead of print 27 June 2019. DOI: 10.1177/2040620719860025.
Google Scholar | Crossref

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