Results 31 to 40 of about 16,303 (230)

A Delphi Consensus Approach for Difficult-to-Treat Patients with Severe Hemophilia A without Inhibitors

Journal of Blood Medicine, 2021
Sreenivas P Veeranki,1 Priti Pednekar,1 Marlon Graf,1 Rifat Tuly,1 Michael Recht,2,3 Katharine Batt1 1PRECISIONheor, Los Angeles, CA, USA; 2American Thrombosis and Hemostasis Network, Rochester, NY, USA; 3The Hemophilia Center, Oregon Health & Science ...
Veeranki SP, Pednekar P, Graf M, Tuly R, Recht M, Batt K   +5 more
doaj  

Induction of activated T follicular helper cells is critical for anti-FVIII inhibitor development in hemophilia A mice

Blood Advances, 2019
: The development of neutralizing anti-FVIII antibodies (inhibitors) is a major complication of FVIII protein replacement therapy in patients with hemophilia A (HA).
Weiqing Jing, Juan Chen, Yuanhua Cai, Yingyu Chen, Jocelyn A. Schroeder, Bryon D. Johnson, Weiguo Cui, Qizhen Shi   +7 more
doaj   +1 more source

Effects of FVIII immunity on hepatocyte and hematopoietic stem cell–directed gene therapy of murine hemophilia A

Molecular Therapy: Methods & Clinical Development, 2016
Immune responses to coagulation factors VIII (FVIII) and IX (FIX) represent primary obstacles to hemophilia treatment. Previously, we showed that hematopoietic stem cell (HSC) retroviral gene therapy induces immune nonresponsiveness to FVIII in both ...
Allison M Lytle, Harrison C Brown, Na Yoon Paik, Kristopher A Knight, J Fraser Wright, H Trent Spencer, Christopher B Doering   +6 more
doaj   +1 more source

The IgG-degrading enzyme, Imlifidase, restores the therapeutic activity of FVIII in inhibitor-positive hemophilia A mice

Haematologica, 2023
Neutralizing anti-factor VIII (FVIII) antibodies, known as FVIII inhibitors, represent a major drawback of replacement therapy in persons with congenital hemophilia A (PwHA), rendering further infusions of FVIII ineffective.
Melissa Bou-Jaoudeh, Sandrine Delignat, Victoria Daventure, Jan Astermark, Hervé Lévesque, Jordan D. Dimitrov, Claire Deligne, Valérie Proulle, Sébastien Lacroix-Desmazes   +8 more
doaj   +1 more source

Potentiation of thrombin generation in hemophilia A plasma by coagulation factor VIII and characterization of antibody-specific inhibition. [PDF]

PLoS ONE, 2012
Development of inhibitory antibodies to coagulation factor VIII (fVIII) is the primary obstacle to the treatment of hemophilia A in the developed world.
Bhavya S Doshi, Bagirath Gangadharan, Christopher B Doering, Shannon L Meeks   +3 more
doaj   +1 more source

TCEP-mediated reduction of a selection of FVIII products.

, 2022
A selection of FVIII products were incubated with PBS or reduced with 1 mM TCEP and free -SH groups visualised with the Alexa Fluor 488-maleimide fluorescent label.
Sanj Raut (12054857), Clive Metcalfe (538890), Andrea Arsiccio (4356397), Carmen Coxon (5902691), Roberto Pisano (4356394)   +4 more
core   +1 more source

Storage of factor VIII variants with impaired von Willebrand factor binding in Weibel-Palade bodies in endothelial cells. [PDF]

PLoS ONE, 2011
BACKGROUND: Point mutations resulting in reduced factor VIII (FVIII) binding to von Willebrand factor (VWF) are an important cause of mild/moderate hemophilia A. Treatment includes desmopressin infusion, which concomitantly increases VWF and FVIII plasma
Maartje van den Biggelaar, Eveline A M Bouwens, Jan Voorberg, Koen Mertens   +3 more
doaj   +1 more source

Suppression of FVIII-Specific Memory B Cells by Chimeric BAR Receptor-Engineered Natural Regulatory T Cells

Frontiers in Immunology, 2020
Anti-drug antibody formation poses tremendous obstacles for optimal treatment of hemophilia A (HA). In this study, we sought to utilize chimeric receptor-modified natural regulatory T cells (Tregs) to target FVIII-specific memory B cells, which are ...
Alessandra De Paula Pohl, Shivaprasad H. Venkatesha, Ai-Hong Zhang, David W. Scott   +3 more
doaj   +1 more source

FVIII inhibitors: pathogenesis and avoidance [PDF]

Blood, 2015
Abstract 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 formation of these antibodies have been performed with the aim of improving the ability to both predict and prevent their appearance.
openaire   +2 more sources

Pathophysiology of bleeding diathesis in haemophilia-A: A sequential and critical appraisal of non-FVIII related haemostatic dysfunctions and their therapeutic implications

Egyptian Journal of Medical Human Genetics, 2018
Haemophilia-A is characterized by deficiency of FVIII, but the bleeding diathesis is not a mere reflection low FVIII activity. The pathophysiology of haemophilic bleeding diathesis is a complex interplay between defective procoagulant function and up ...
Umma A. Ibrahim, Sagir G. Ahmed
doaj   +1 more source