title: Acquired hemophilia a: a current review of autoantibody disease.
abstract: Acquired hemophilia A (AHA) is a rare, potentially life-threatening hemorrhagic disorder that presents a complex clinical challenge. The immune-mediated production of autoantibodies, known as factor VIII inhibitors, often results in clinically significant soft tissue or post-procedural bleeding episodes in patients without a previous diagnosis of a bleeding disorder. Acquired antibodies against factor VIII are associated with an extensive list of conditions, including pregnancy, autoimmune disease, and malignancy. There is great potential for morbidity and mortality resulting from autoantibody development. Death is more frequent within the first few weeks after symptomatic manifestation, making prompt recognition and treatment vitally important. Treatment focuses on stabilization of initial bleeding and long-term eradication of the acquired inhibitor. As no randomized clinical trials have been conducted regarding treatment in this patient population, clinical expertise and experience continue to guide treatment recommendations. This report provides an algorithm for the diagnosis of AHA and outlines potential treatment recommendations, most notably concomitant use of recombinant factor VIIa (rF7a) and factor VIII inhibitor bypassing agent to control bleeding in patients not responsive to single-agent therapy, and use of rituximab and prednisone for inhibitor eradication therapy.
authors.affiliation: Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, Utah 84132, USA. douglas.sborov@hsc.utah.edu
authors.forename: Douglas W
authors.initials: DW
authors.lastname: Sborov
authors.forename: George M
authors.initials: GM
authors.lastname: Rodgers
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D005169:Factor VIII / Q000276:immunology; D006467:Hemophilia A / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D006801:Humans; D008297:Male
chemical_list: D001323:Autoantibodies; D005169:Factor VIII