Risk factors for inhibitor development in severe hemophilia A

I Garagiola, R Palla, F Peyvandi - Thrombosis research, 2018 - Elsevier
Although significant advances in hemophilia treatment have improved patient outcomes and
quality of life, one of the greatest complications in severe hemophilia A is the development of …

Biological considerations of plasma-derived and recombinant factor VIII immunogenicity

J Lai, C Hough, J Tarrant… - Blood, The Journal of the …, 2017 - ashpublications.org
In hemophilia A, the most severe complication of factor VIII (FVIII) replacement therapy
involves the formation of FVIII neutralizing antibodies, also known as inhibitors, in 25% to …

The endothelial cell receptor stabilin-2 regulates VWF-FVIII complex half-life and immunogenicity

LL Swystun, JD Lai, C Notley… - The Journal of …, 2018 - Am Soc Clin Investig
Quantitative abnormalities of the von Willebrand factor–factor VIII (VWF-FVIII) complex
associate with inherited bleeding or thrombotic disorders. Receptor-mediated interactions …

Factor VIII trafficking to CD4+ T cells shapes its immunogenicity and requires several types of antigen-presenting cells

R Kaczmarek, AR Piñeros, PE Patterson… - Blood, The Journal …, 2023 - ashpublications.org
Despite> 80 years of clinical experience with coagulation factor VIII (FVIII) inhibitors,
surprisingly little is known about the in vivo mechanism of this most serious complication of …

Marginal zone B cells are critical to factor VIII inhibitor formation in mice with hemophilia A

PE Zerra, C Cox, WH Baldwin, SR Patel… - Blood, The Journal …, 2017 - ashpublications.org
Although factor VIII (FVIII) replacement therapy can be lifesaving for patients with hemophilia
A, neutralizing alloantibodies to FVIII, known as inhibitors, develop in a significant number of …

[HTML][HTML] Product type and other environmental risk factors for inhibitor development in severe hemophilia A

F Peyvandi, I Garagiola - Research and Practice in Thrombosis and …, 2018 - Elsevier
Essentials• Treatment complications in hemophilia A: inhibitors development.• Type of FVIII
products: the latest updates on plasma‐derived or recombinant FVIII immunogenicity.• …

Vaccinations are not associated with inhibitor development in boys with severe haemophilia A

H Platokouki, K Fischer, SC Gouw, A Rafowicz… - …, 2018 - Wiley Online Library
Background Inhibitor development in previously untreated patients (PUP s) with severe
haemophilia A is a multifactorial event. It is unknown whether paediatric vaccinations given …

Non‐genetic risk factors in haemophilia A inhibitor management–the danger theory and the use of animal models

KM Lövgren, H Søndergaard, S Skov… - Haemophilia, 2016 - Wiley Online Library
In haemophilia A (HA) management, antidrug antibodies, or inhibitors, are a serious
complication that renders factor VIII (FVIII) replacement therapy ineffective, increases …

[HTML][HTML] N-linked glycosylation modulates the immunogenicity of recombinant human factor VIII in hemophilia A mice

JD Lai, LL Swystun, D Cartier, K Nesbitt, C Zhang… - …, 2018 - ncbi.nlm.nih.gov
Immune responses to factor VIII remain the greatest complication in the treatment of severe
hemophilia A. Recent epidemiological evidence has highlighted that recombinant factor VIII …

An update on the 'danger theory'in inhibitor development in hemophilia A

SJ Schep, M Boes, REG Schutgens… - Expert Review of …, 2019 - Taylor & Francis
Introduction: Nowadays, one of the most serious treatment complications in hemophilia A is
the formation of neutralizing antibodies against coagulation factor VIII (FVIII). These so …