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Case Reports
. 2023 Jul 10;12(14):4590.
doi: 10.3390/jcm12144590.

Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review

Carola Sella  1   2   3 , Marco Bardetta  1   2   3 , Federica Valeri  1   2 , Cristina Dainese  1   2 , Alessandra Valpreda  4 , Massimo Massaia  5 , Daniele Grimaldi  5 , Annamaria Porreca  6 , Benedetto Bruno  2   3 , Alessandra Borchiellini  1   2
Affiliations
Case Reports

Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review

Carola Sella et al. J Clin Med. .

Abstract

Background: Acquired hemophilia A (AHA) is a rare bleeding disease due to autoantibodies directed against clotting factor VIII (FVIII). Treatment of AHA consists of inhibitor eradication with immunosuppressive therapy (IST) and prompt control of bleeding obtained with bypassing agents or recombinant porcine FVIII (rpFVIII). The latter has recently been licensed for management of acute bleeding in AHA. Unlike treatment with bypassing agents, rpFVIII can be monitored to provide a successful hemostatic effect and avoid overtreatment. Correlation between rpFVIII inhibitor titers and efficacy of rpFVIII treatment remains a matter of debate.

Methods: We report three cases of AHA in which rpFVIII was successfully used with an unconventional schedule despite the presence of medium-high titers of the rpFVIII. The modified Nijmegen-Bethesda inhibitor assay (NBA) was used to dose porcine FVIII inhibitors.

Result: The presence of rpFVIII inhibitors prior to the exposition to susoctocog-alfa, that may suggest a cross-reactivity with human FVIII inhibitors, did not affect hemostasis.

Conclusion: In our experience, rpFVIII demonstrates safety and efficacy in the presence of rpFVIII inhibitors and using an unconventional schedule in both the perioperative and outpatient settings. Laboratory measurement of inhibitors against rpFVIII during treatment is described for the first time.

Keywords: acquired hemophilia A; prophylaxis; recombinant porcine FVIII; surgery.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Factor VIII (FVIII) activity (%) and human factor VIII (hFVIII) inhibitors titer (UB/mL) during rpFVIII infusion.
See this image and copyright information in PMC

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