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

Author:

Sella Carola123,Bardetta Marco123,Valeri Federica12,Dainese Cristina12,Valpreda Alessandra4,Massaia Massimo5,Grimaldi Daniele5,Porreca Annamaria6,Bruno Benedetto23ORCID,Borchiellini Alessandra12ORCID

Affiliation:

1. Regional Centre for Hemorrhagic and Thrombotic Diseases, AOU Città della Salute e della Scienza, 10126 Turin, Italy

2. Division of Hematology, AOU Città della Salute e della Scienza, University of Turin, 10126 Turin, Italy

3. Department of Molecular Biotechnology and Health Sciences, University of Turin, 10124 Turin, Italy

4. Central Laboratory Baldi and Riberi, AOU Città della Salute e della Scienza, University of Turin, 10124 Turin, Italy

5. Hematology Unit, Santa Croce e Carle Hospital, 12100 Cuneo, Italy

6. Department of Medical, Oral and Biotechnologies Science, University of Chieti-Pescara, 66100 Chieti, Italy

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.

Publisher

MDPI AG

Subject

General Medicine

Reference32 articles.

1. Acquired hemophilia A: Updated review of evidence and treatment guidance;Kempton;Am. J. Hematol.,2017

2. Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding;Coppola;Blood Transfus.,2022

3. A case report of acquired hemophilia following COVID-19 vaccine;Radwi;J. Thromb. Haemost.,2021

4. The first case of acquired hemophilia A associated with SARS-CoV-2 infection;Franchini;Am. J. Hematol.,2020

5. Acquired hemophilia A in the United Kingdom: A 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation;Collins;Blood,2006

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3