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 …

FVIII inhibitors: pathogenesis and avoidance

J Astermark - Blood, The Journal of the American Society of …, 2015 - ashpublications.org
The pathogenesis of inhibitory antibodies has been the focus of major scientific interest over
the last decades, and several studies on underlying immune mechanisms and risk factors for …

Differential effects of bleeds on the development of arthropathy–basic and applied issues

LFD Van Vulpen, SC Mastbergen, F Lafeber… - …, 2017 - Wiley Online Library
Haemophilia is characterized by a spontaneous bleeding tendency, affecting mainly the
synovial joints. Recurrent joint bleeds induce a cascade of inflammatory as well as …

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 …

Tolerogenic properties of the Fc portion of IgG and its relevance to the treatment and management of hemophilia

RS Blumberg, D Lillicrap… - Blood, The Journal …, 2018 - ashpublications.org
Hemophilia, or inherited genetic deficiencies in coagulation factors, results in uncontrolled
bleeding requiring replacement therapy with recombinant proteins given preventively or on …

Recombinant factor VIII Fc fusion protein drives regulatory macrophage polarization

K Kis-Toth, GM Rajani, A Simpson, KL Henry… - Blood …, 2018 - ashpublications.org
The main complication of replacement therapy with factor in hemophilia A (HemA) is the
formation of inhibitors (neutralizing anti–factor VIII [FVIII] antibodies) in∼ 30% of severe …

To serve and protect: the modulatory role of von Willebrand factor on factor VIII immunogenicity

RB Hartholt, AS van Velzen, I Peyron, A Ten Brinke… - Blood reviews, 2017 - Elsevier
Hemophilia A is a bleeding disorder characterized by the absence or dysfunction of blood
coagulation factor VIII (FVIII). Patients are treated with regular infusions of FVIII concentrate …

Risk stratification integrating genetic data for factor VIII inhibitor development in patients with severe hemophilia A

D Bachelet, T Albert, C Mbogning, S Hässler, Y Zhang… - PLoS …, 2019 - journals.plos.org
Replacement therapy in severe hemophilia A leads to factor VIII (FVIII) inhibitors in 30% of
patients. Factor VIII gene (F8) mutation type, a family history of inhibitors, ethnicity and …

Humanized cobra venom factor: structure, activity, and therapeutic efficacy in preclinical disease models

CW Vogel, PW Finnegan, DC Fritzinger - Molecular Immunology, 2014 - Elsevier
The complement system is an integral component of both innate and adaptive immunity.
However, complement is also a pathogenetic factor in many diseases. The development of …

Inhibitors—recent insights

KP Pratt, VR Arruda, S Lacroix‐Desmazes - Haemophilia, 2021 - Wiley Online Library
The development of inhibitory antibodies to therapeutic factor VIII (FVIII) in haemophilia A
(HA) patients is the major complication in treatment/prevention of haemorrhages. The …