Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII?

Abstract

Acquired hemophilia A (AHA) is caused by autoantibodies neutralizing coagulation factor VIII (FVIII). In the presence of inhibitors against FVIII, acute bleeds can be managed with bypassing agents, including recombinant factor VIIa (eptacog alfa activated, NovoSeven) and activated prothrombin complex concentrate (FEIBA), as well as recombinant porcine FVIII (susoctocog alfa, Obizur). Studies comparing these agents directly are not available, and indirect evidence suggests an overall similar efficacy. Selecting an agent in clinical practice therefore depends on (1) availability of agent, (2) safety profile, (3) monitoring requirements, (4) cost, and (5) personal experience. This review examines available data and collects additional considerations to support decision making for bleeding emergencies in AHA.

Zusammenfassung

Die erworbene Hämophilie A wird durch neutralisierende Autoantikörper gegen den Gerinnungsfaktor VIII (FVIII) verursacht. In der Gegenwart von FVIII-Inhibitoren werden akute Blutungen mit sogenannten Bypass-Produkten, rekombinantem Faktor VIIa (NovoSeven®) und aktiviertem Prothrombinkomplex-Konzentrat (FEIBA®), sowie rekombinantem porcinem FVIII (Susoctocogalfa, Obizur®) behandelt. Direkt vergleichende Studien liegen nicht vor und indirekte Vergleich legen ähnliche Effektivität nahe. Die Auswahl eines Medikaments zur Behandlung von Blutungen beruht deshalb vor allem auf (i) seiner kurzfristigen Verfügbarkeit, (ii) seinem Sicherheitsprofil, (iii) Anforderungen an das Labormonitoring, (iv) Kosten und (v) persönlicher Erfahrung. Dieser Artikel trägt die verfügbaren Daten und weitere Überlegungen zusammen, um die Entscheidungsfindung in Blutungsnotfällen bei erworbener Hämophilie zu unterstützen.

Keywords

factor VIII - inhibitor - recombinant factor VIIa - activated prothrombin complex concentrate

Schlüsselwörter

Faktor VIII - Inhibitor - rekombinanter Faktor VIIa - aktiviertes Prothrombinkomplex-Konzentrat

Publication History

Received: 18 March 2020

Accepted: 05 May 2020

Publication Date:
11 September 2020 (online)

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