Vincent P Diego, Bernadette W Luu, Marco Hofmann, Long V Dinh, Marcio Almeida, Jerry S Powell, Raja Rajalingam, Juan M Peralta, Satish Kumar, Joanne E Curran, Zuben E Sauna, Roberta Kellerman, Yara Park, Nigel S Key, Miguel A Escobar, Huy Huynh, Anne M Verhagen, Sarah Williams-Blangero, Paul V Lehmann, Eugene Maraskovsky, John Blangero, Tom E Howard
BACKGROUND: Plasma-derived (pd) or recombinant (r) therapeutic FVIII proteins (FVIIIs) are infused to arrest/prevent bleeding in patients with hemophilia A (PWHA). However, FVIIIs are neutralized if anti-FVIII-antibodies ("inhibitors") develop. Accumulating evidence suggests that pdFVIIIs with von Willebrand factor (VWF) are less immunogenic than rFVIIIs and that distinct rFVIIIs are differentially immunogenic. Since inhibitor development is T-helper-cell-dependent, HLA-class-II (HLAcII) molecules comprise an important early determinant...
September 25, 2019: Journal of Thrombosis and Haemostasis: JTH