Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients. Issue 3 (April 2019)

• Record Type: Journal Article
• 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
• Is Part Of: Blood coagulation and fibrinolysis. Volume 30:Issue 3(2019)
• Publication Date: 2019-04
• Subjects: autoantibodies -- coagulation protein disorders -- factor VIII deficiency -- hemorrhage -- immunosuppression; Blood -- Coagulation -- Periodicals; Fibrinolysis -- Periodicals; Hemostasis -- Periodicals; Thrombosis -- Periodicals; Blood Coagulation -- Periodicals; Fibrinolysis -- Periodicals; Hemostasis -- Periodicals; Thrombosis -- Periodicals; 612.115
• DOI: 10.1097/MBC.0000000000000804
• Languages: English
• ISSNs: 0957-5235

Abstract

Abstract : N/A: 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.