Review of Potential Barriers to Effective Hemostatic Management of Acquired Hemophilia A by Non-Hemophilia Experts in the United States

Abstract

Acquired hemophilia A (AHA) is an ultra-rare autoimmune disorder caused by autoantibodies against factor VIII. It often presents with life-threatening bleeding to non-hemophilia experts, who have limited awareness of this condition. This review evaluated hemostatic management and identified barriers to optimal management of AHA by non-hemophilia experts in the United States through a literature review. AHA case reports published by non-hemophilia experts from January 2016 through November 2021 in nonhematology journals were critically reviewed for a chronology of clinical course and management, consultation with a hemophilia expert, referencing of available AHA recommendations, discussion of all hemostatic options, and bleed control outcomes; 24 case reports representing 24 patients were identified. Twelve patients had an apparent delay in diagnosis, 17 cases did not seek expert consultation, and 15 did not reference the 2009 International AHA Recommendations, including six in whom hemostatic treatment was not consistent with the recommendations. Of the 17 articles published after the 2017 AHA Guidance, eight did not reference them. Of the five articles published after the 2020 International Recommendations for AHA, three did not reference them. Overall, 14 articles did not discuss all available hemostatic treatment options. Four patients died. Our findings reveal variability in hemostatic management of AHA by nonhemophilia experts in the United States. Lack of AHA awareness remains a primary barrier for optimal management of AHA among non-hemophilia experts. Increasing education about existing AHA guidelines, including available therapies and access to expert care at hemophilia treatment centers, may help improve the outcomes of patients with AHA.

Recombinant porcine factor VIII in patients with congenital haemophilia A with inhibitors undergoing surgery: Phase 3, single‐arm, open‐label study

“…This was based on rpFVIII sharing sufficient sequence homology with hFVIII to be haemostatic but being different enough to be less susceptible to inactivation by circulating FVIII inhibitors. 21 In addition, these patients have a clear clinical need for rpFVIII as they are a difficult-to-treat group. In this surgery study, good haemostasis was achieved with rpFVIII during the immediate perioperative period, but 5 of the 8 patients experienced anamnestic reactions.…”

“…At the initiation of this study, it was expected that the benefit‐risk ratio would favour the short‐term use of rpFVIII in patients with CHAWI undergoing surgical or invasive procedures. This was based on rpFVIII sharing sufficient sequence homology with hFVIII to be haemostatic but being different enough to be less susceptible to inactivation by circulating FVIII inhibitors 21 . In addition, these patients have a clear clinical need for rpFVIII as they are a difficult‐to‐treat group.…”