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Reappearance of inhibitor in a tolerized patient with severe haemophilia A during FVIII‐free emicizumab therapy

Abstract : Introduction: The recent approval of emicizumab has significantly changed the management of severe hemophilia A. It also raises several questions, particularly concerning the need to maintain FVIII infusion in emicizumab-treated patients after successful ITI. Indeed, the possible reappearance of the inhibitor is a major concern, while, to date, no recurrence of the inhibitor has been observed in successfully tolerized patients. Aim: To describe the reappearance of an inhibitor in a successfully tolerized patient with severe hemophilia A during emicizumab therapy alone (in the absence of any exposure to FVIII), and to discuss the benefit in maintaining FVIII infusion. Methods: Case report Results: We report on a case in which inhibitor production spontaneously reappeared more than 7 years after successful ITI and 4 months after emicizumab initiation and FVIII withdrawal. Indeed, the patient was inhibitor-negative one month after emicizumab initiation and despite the absence of any FVIII infusions, a low positive titer was observed 4 months later together with reemergence of both anti-FVIII IgG1 and IgG4. Conclusion: Our observation supports the hypothesis whereby regular exposure to FVIII may be necessary to maintain successful, specific immune tolerance. Therefore, emicizumab-treated patients with a history of inhibitor, even those not exposed to FVIII, should be regularly screened for the presence of an inhibitor.
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https://hal.archives-ouvertes.fr/hal-03431426
Contributor : Sébastien Lacroix-Desmazes Connect in order to contact the contributor
Submitted on : Thursday, May 12, 2022 - 10:26:49 AM
Last modification on : Saturday, May 14, 2022 - 3:12:23 AM

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Ladislas Capdevila, Delphine Borgel, Dominique Lasne, Sébastien Lacroix-Desmazes, Maximilien Desvages, et al.. Reappearance of inhibitor in a tolerized patient with severe haemophilia A during FVIII‐free emicizumab therapy. Haemophilia, Wiley, 2021, 27 (4), pp.e581-e584. ⟨10.1111/hae.14334⟩. ⟨hal-03431426⟩

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