• Sally Campbell, Jane Mason, Ritam Prasad, Harriet Ambrose, Stewart Hunt, and Huyen Tran.
• Haemophilia Treatment Centre, Alfred Hospital, Melbourne, Victoria, Australia.
• Intern Med J. 2021 Feb 1; 51 (2): 215-219.

BackgroundAcquired haemophilia A (AHA) is a rare acquired bleeding disorder that can present with life-threatening bleeding.AimsTo describe recent Australian use of recombinant porcine factor VIII (rpFVIII) replacement therapy as a haemostatic agent in patients with acquired haemophilia.MethodsFour patients with acquired haemophilia treated in three different institutions around Australia in the past 12 months were included in the study. Haemostatic efficacy of Obizur (Takeda) was assigned by the treating haematologist according to previously published criteria.ResultsSix bleeds were treated with rpFVIII, three of which were initially refractory to treatment with recombinant VIIa. rpFVIII was rated efficacious in 100% of bleeds by 24 h. rpFVIII loading dose was 100 U/kg (100-120 U kg-1 ) and this increased the factor VIII level (via one-stage FVIII assay) from <1-1.2% to 54-306% taken 0.5-1.5 h post-infusion. Subsequent doses ranged from 40 to 60 U/kg twice daily or daily for 3 to 13 days. No rpFVIII related adverse events occurred. Three of the four patients achieved complete remission and were weaned from immunosuppression. One patient died prior to achieving partial remission, secondary to an arterial ischaemic event.ConclusionThis case series demonstrates that recombinant porcine FVIII is efficacious to treat acute bleeds in acquired haemophilia, including in those who are refractory to bypassing agents. Doses of rpFVIII were able to be titrated based on FVIII level and clinical response.© 2020 Royal Australasian College of Physicians.

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