Emicizumab for the Treatment of Acquired Hemophilia A: Consensus Recommendations from the GTH-AHA Working Group

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • Christian Pfrepper - , Universitätsklinikum Leipzig (Autor:in)
  • Robert Klamroth - , Vivantes Klinikum im Friedrichshain (Autor:in)
  • Johannes Oldenburg - , Universitätsklinikum Bonn, Deutsches Zentrum für Neurodegenerative Erkrankungen e.V. (DZNE) (Autor:in)
  • Katharina Holstein - , Universitätsklinikum Hamburg-Eppendorf (UKE) (Autor:in)
  • Hermann Eichler - , Universitätsklinikum des Saarlandes (Autor:in)
  • Christina Hart - , Universitätsklinikum Regensburg (Autor:in)
  • Patrick Moehnle - , Klinikum der Ludwig-Maximilians-Universität (LMU) München (Autor:in)
  • Kristina Schilling - , Universitätsklinikum Jena (Autor:in)
  • Karolin Trautmann-Grill - , Medizinische Klinik und Poliklinik I (Autor:in)
  • Mohammed Alrifai - , Universitätsklinikum Gießen und Marburg GmbH (Autor:in)
  • Cihan Ay - , Medizinische Universität Wien (Autor:in)
  • Wolfgang Miesbach - , Johann Wolfgang Goethe-Universität Frankfurt am Main (Autor:in)
  • Paul Knoebl - , Medizinische Universität Wien (Autor:in)
  • Andreas Tiede - , Medizinische Hochschule Hannover (MHH) (Autor:in)

Abstract

BACKGROUND:  Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Standard treatment consists of bleeding control with bypassing agents and immunosuppressive therapy. Emicizumab is a bispecific antibody that mimics the function of activated FVIII irrespective of the presence of neutralizing antibodies. Recently, the GTH-AHA-EMI study demonstrated that emicizumab prevents bleeds and allows to postpone immunosuppression, which may influence future treatment strategies.

AIM:  To provide clinical practice recommendations on the use of emicizumab in AHA.

METHODS:  A Delphi procedure was conducted among 33 experts from 16 German and Austrian hemophilia care centers. Statements were scored on a scale of 1 to 9, and agreement was defined as a score of ≥7. Consensus was defined as ≥75% agreement among participants, and strong consensus as ≥95% agreement.

RESULTS:  Strong consensus was reached that emicizumab is effective for bleed prophylaxis and should be considered from the time of diagnosis (100% consensus). A fast-loading regimen of 6 mg/kg on day 1 and 3 mg/kg on day 2 should be used if rapid bleeding prophylaxis is required (94%). Maintenance doses of 1.5 mg/kg once weekly should be given (91%). Immunosuppression should be offered to patients on emicizumab if they are eligible based on physical status (97%). Emicizumab should be discontinued when remission of AHA is achieved (97%).

CONCLUSION:  These GTH consensus recommendations provide guidance to physicians on the use of emicizumab in AHA and follow the results of clinical trials that have shown emicizumab is effective in preventing bleeding in AHA.

Details

OriginalspracheEnglisch
FachzeitschriftHamostaseologie
PublikationsstatusElektronische Veröffentlichung vor Drucklegung - 4 Dez. 2023
Peer-Review-StatusJa

Externe IDs

Scopus 85179694533

Schlagworte