title: The tipping point: The critical role of therapeutic apheresis in a case of refractory acquired hemophilia.
abstract: Acquired hemophilia A (AHA) is a rare autoimmune disorder that leads to factor VIII (FVIII) deficiency via autoantibody formation. Standard treatment options include FVIII bypassing factors and immunosuppression. However, the role of therapeutic plasma exchange (TPE) is not clear in the treatment of AHA. We present a case of idiopathic AHA in a 66 year old female with severe bleeding and a FVIII inhibitor of 17.6 Bethesda units (BU). She failed to respond to standard treatment including maximum dose of recombinant FVIIa (rFVIIa), rituximab, and other immunosuppressive agents. Her FVIII inhibitor rapidly increased to 140 BU and FVIII was below 5%. TPE was initiated 3 weeks after admission and her bleeding stabilized after the first treatment and completely stopped after three treatments. Repeat testing revealed increased FVIII to 15% and FVIII inhibitor decreased to 2.0 BU. After an additional TPE treatment, her FVIII increased to 27% and FVIII inhibitor decreased to 0.6 BU and she was discharged without bleeding 40 days after admission. In this case, TPE played a critical role in reducing FVIII inhibitor, which resulted in a recovery of FVIII activity and hemostasis. Therefore, TPE should be initiated early in AHA patients with bleeding and high titer of FVIII inhibitor.
authors.affiliation: Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.|Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Losos
authors.affiliation: Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
authors.forename: Scott
authors.initials: S
authors.lastname: Scrape
authors.affiliation: Department of Hematology, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Sarita
authors.initials: S
authors.lastname: Joshi
authors.affiliation: Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Shmookler
authors.affiliation: Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
authors.forename: Jian
authors.initials: J
authors.lastname: Chen
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000302:isolation & purification; D005169:Factor VIII / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000628:therapy*; D006470:Hemorrhage / Q000628:therapy; D006801:Humans; D010951:Plasma Exchange / Q000379:methods*; D016879:Salvage Therapy
chemical_list: D001323:Autoantibodies; C078147:F8 protein, human; D005169:Factor VIII
keywords: acquired hemophilia A; factor VIII inhibitor; therapeutic apheresis