Journal of Blood Medicine (May 2022)
Acquired Hemophilia A: Current Guidance and Experience from Clinical Practice
Abstract
Allyson M Pishko,1 Bhavya S Doshi2 1Department of Medicine, Division of Hematology/Oncology, University of Pennsylvania, Philadelphia, PA, USA; 2Department of Pediatrics, Division of Hematology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USACorrespondence: Bhavya S Doshi, Department of Pediatrics, Division of Hematology, Children’s Hospital of Philadelphia, 3501 Civic Center Blvd, Colket Translational Research Building Rm 5024, Philadelphia, PA, 19104, USA, Tel +1 215-590-3437, Fax +1 215-590-3992, Email [email protected]: In acquired hemophilia A (AHA), autoantibodies to coagulation factor VIII (FVIII) neutralize FVIII activity leading to a potentially severe bleeding diathesis that carries a high rate of morbidity and mortality. This disorder is rare and occurs mainly in adults over 60 years of age or in the postpartum period. The diagnosis should be suspected in patients with new-onset bleeding without a personal or family history of bleeding and can be confirmed via specific assays for FVIII inhibitors. Treatment involves both hemostatic therapies to decrease bleeding and immune modulation strategies to re-establish immune tolerance to FVIII. There are limited data on treatment for refractory disease, based mostly on small case series. Registry studies have informed consensus guidelines for optimal hemostatic therapies and initial immunosuppressive therapies. Additional studies are needed to evaluate novel hemostatic agents and develop biomarkers to risk-stratify treatment while limiting adverse events.Keywords: factor VIII, autoantibodies, hemostasis, immune modulation
