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

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...

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Autores principales: Sella, Carola, Bardetta, Marco, Valeri, Federica, Dainese, Cristina, Valpreda, Alessandra, Massaia, Massimo, Grimaldi, Daniele, Porreca, Annamaria, Bruno, Benedetto, Borchiellini, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10380857/
https://www.ncbi.nlm.nih.gov/pubmed/37510704
http://dx.doi.org/10.3390/jcm12144590
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author Sella, Carola
Bardetta, Marco
Valeri, Federica
Dainese, Cristina
Valpreda, Alessandra
Massaia, Massimo
Grimaldi, Daniele
Porreca, Annamaria
Bruno, Benedetto
Borchiellini, Alessandra
author_facet Sella, Carola
Bardetta, Marco
Valeri, Federica
Dainese, Cristina
Valpreda, Alessandra
Massaia, Massimo
Grimaldi, Daniele
Porreca, Annamaria
Bruno, Benedetto
Borchiellini, Alessandra
author_sort Sella, Carola
collection PubMed
description 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.
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spelling pubmed-103808572023-07-29 Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review Sella, Carola Bardetta, Marco Valeri, Federica Dainese, Cristina Valpreda, Alessandra Massaia, Massimo Grimaldi, Daniele Porreca, Annamaria Bruno, Benedetto Borchiellini, Alessandra J Clin Med Case Report 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. MDPI 2023-07-10 /pmc/articles/PMC10380857/ /pubmed/37510704 http://dx.doi.org/10.3390/jcm12144590 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Sella, Carola
Bardetta, Marco
Valeri, Federica
Dainese, Cristina
Valpreda, Alessandra
Massaia, Massimo
Grimaldi, Daniele
Porreca, Annamaria
Bruno, Benedetto
Borchiellini, Alessandra
Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title_full Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title_fullStr Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title_full_unstemmed Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title_short Surgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review
title_sort surgery and prophylaxis with susoctocog-alfa in acquired hemophilia: case series and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10380857/
https://www.ncbi.nlm.nih.gov/pubmed/37510704
http://dx.doi.org/10.3390/jcm12144590
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