Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients

• Published in: Blood coagulation & fibrinolysis Vol. 30; no. 3; pp. 127 - 132
• Main Authors: Mingot-Castellano, María E, Moret, Andrés, de Cos, Carmen, García-Candel, Faustino, Garrido, Raquel, González-Porras, José R, López-Fernández, María F, Quintana, Laura, Rodríguez-González, Ramón, Marco, Pascual
• Format: Journal Article
• Language: English
• Published: England Copyright YEAR Wolters Kluwer Health, Inc. All rights reserved 01-04-2019
• Subjects: Antibody Specificity; Autoantibodies - immunology; Factor VIII - immunology; Hemophilia A - drug therapy; Humans; Immunosuppressive Agents - therapeutic use; Prospective Studies; Protein Domains; Treatment Outcome
• ISSN: 0957-5235; 1473-5733
• DOI: 10.1097/MBC.0000000000000804

We hypothesized that inhibitor specificity may predict the outcome of antifactor VIII autoantibodies eradication treatment in acquired hemophilia A. Our objective was to analyze the association between factor VIII domains recognized by inhibitors and outcome of the immunosuppressive therapies (ISTs) in a prospective, observational study. 16 patients were recruited. Inhibitor specificities were assessed at diagnosis and throughout the study. Their association with IST outcome was addressed. First-line IST succeeded in 56% of patients. Inhibitors reacted mainly with light chain domains (69%) and/or the A2 domain (44%). 31% inhibitors recognized more than one domain. Significantly, the number of patients whose inhibitors recognized the light chain was significantly higher in the group of those who did not reach complete remission after first line IST when compared with those who did [6/7 (85.7%) vs. 4/9 (44.4%), P < 0.05]. Therefore, inhibitor specificity could predict the success of IST in acquired hemophilia A.