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Case Reports
. 2022 Mar 27;6(2):e12688.
doi: 10.1002/rth2.12688. eCollection 2022 Feb.

Recombinant porcine factor VIII in acquired hemophilia A: Experience from two patients and literature review

Alexander Hayden  1 , Nellowe Candelario  1 , Genevieve Moyer  1   2
Affiliations
Case Reports

Recombinant porcine factor VIII in acquired hemophilia A: Experience from two patients and literature review

Alexander Hayden et al. Res Pract Thromb Haemost. .

Abstract

Background: Acquired hemophilia A (AHA) is a disease caused by antibody formation inhibiting the function of factor VIII, causing bleeding. Recombinant porcine factor VIII (rpFVIII) escapes human FVIII antibody recognition and can provide life-saving hemostasis. However, the development of antibodies against pFVIII can limit its use. We report two cases in which loss of response to rpFVIII occurred, likely because of inhibiting antibodies. In case 1, the patient achieved hemostasis but lost response to rpFVIII within a few days. In the second case, rpFVIII controlled bleeding but the patient experienced diminishing half-life of rpFVIII infusions over time, necessitating a switch to emicizumab which provided lasting hemostasis.

Key clinical question: Based on our experience with these cases, we reviewed the available literature regarding the use of rpFVIII in AHA. The Key Clinical Question was to determine how often inhibitors were associated with rpFVIII treatment failure.

Clinical approach and conclusions: We identified 43 AHA patients across five studies who were treated with rpFVIII. Twenty-two patients (51%) developed pFVIII inhibitors and seven cases (16%) reported loss of efficacy associated with an inhibitor. In conclusion, rpFVIII can be a life-saving therapy in AHA. However, clinicians should be aware that pFVIII antibody development can reduce the efficacy and duration of response. Recombinant pFVIII's limitations support the utility of further investigation of alternative therapies such as emicizumab in early AHA management.

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Figures

FIGURE 1
FIGURE 1
Factor VIII activity over hospital course in days along with timing of rPFVIII doses. Abbreviations: FVIII, factor VIII activity; rPFVIII, recombinant porcine factor VIII
See this image and copyright information in PMC

References

    1. Delgado J, Jimenez‐Yuste V, Hernandez‐Navarro F, Villar A. Acquired haemophilia: review and meta‐analysis focused on therapy and prognostic factors. Br J Haematol. 2003;121(1):21‐35. - PubMed
    1. Collins PW, Hirsch S, Baglin TP, et al. Acquired hemophilia A in the United Kingdom: a 2‐year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood. 2007;109(5):1870‐1877. - PubMed
    1. Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol. 2005;80(1):55‐63. - PubMed
    1. Lossing TS, Kasper CK, Feinstein DI. Detection of factor VIII inhibitors with the partial thromboplastin time. Blood. 1977;49(5):793‐797. - PubMed
    1. Collins P, Baudo F, Knoebl P, et al. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012;120(1):47‐55. - PMC - PubMed

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