Hemostatic therapy as a management strategy for acquired hemophilia: what does the future hold?

Expert Rev Hematol. 2021 Mar;14(3):263-270 doi: 10.1080/17474086.2021.1892483.

Introduction: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies that bind and inactivate factor VIII (FVIII), predisposing to a potentially life-threatening bleeding.Areas covered: The main epidemiological, clinical, laboratory and therapeutic features of AHA are critically discussed. In particular, we focus on the hemostatic management of AHA patients analyzing the currently available treatment options and showing the latest data on the innovative hemostatic agents still under investigation. Authors searched the Medline and PubMed electronic databases for publication on AHA in the last twenty years.Expert opinion: While a rapid recognition of suspected cases of AHA is essential to make a correct diagnosis and appropriately and timely treat the hemorrhagic manifestations, the multidisciplinary approach to this challenging, rare and life-threatening bleeding disorder is of equal importance to improve patients' outcome. Although promising, the safety and efficacy of the clinical use of emicizumab in AHA needs to be validated by trials including an adequate number of patients, before registering the drug also for this indication.

KEYWORDS: Acquired hemophilia A; autoantibodies; bleeding; inhibitors
MESH HEADINGS: Autoantibodies; Autoimmune Diseases; Factor VIII; Hemophilia A; Hemorrhage; Hemostatics; Humans
Study Details
Study Design: Systematic Review
Language: eng
Credits: Bibliographic data from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine