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

Research output: Contribution to journalResearch articleContributedpeer-review


  • Christian Pfrepper - , University Hospital Leipzig (Author)
  • Robert Klamroth - , Vivantes Hospitals Berlin (Author)
  • Johannes Oldenburg - , University of Bonn Medical Center, German Center for Neurodegenerative Diseases (DZNE) (Author)
  • Katharina Holstein - , University Hospital Hamburg Eppendorf (Author)
  • Hermann Eichler - , University Hospital of Saarland (Author)
  • Christina Hart - , University Hospital Regensburg (Author)
  • Patrick Moehnle - , Hospital of the Ludwig-Maximilians-University (LMU) Munich (Author)
  • Kristina Schilling - , Jena University Hospital (Author)
  • Karolin Trautmann-Grill - , Department of internal Medicine I (Author)
  • Mohammed Alrifai - , University Hospital Gießen and Marburg (Author)
  • Cihan Ay - , Medical University of Vienna (Author)
  • Wolfgang Miesbach - , Goethe University Frankfurt a.M. (Author)
  • Paul Knoebl - , Medical University of Vienna (Author)
  • Andreas Tiede - , Hannover Medical School (MHH) (Author)


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.


Original languageEnglish
Number of pages6
Publication statusE-pub ahead of print - 4 Dec 2023

External IDs

Scopus 85179694533


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