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 non-hematology 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 non-hemophilia 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.

Keywords: acquired hemophilia a; acquired hemophilia a management; diagnosis; fviii autoantibodies; treatment practices.

Figure 1. Literature search strategy flow chart

AHA, acquired hemophilia A.

Figure 2. Overlap between a lack of hemophilia-expert consultation, discussion of treatment options, and apparent delay in diagnosis in 24 AHA case reports published by non-hemophilia-expert authors.

*Three studies [16,17,26]. †Four studies [13,14,18,23]. ‡Five studies [21,27-30]. §Four studies [12,15,19,22]. ǁOne study [31]. ¶Four studies [24,25,32,33]. #Three studies [20,34,35]. n: the number of studies; AHA: acquired hemophilia A