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- Title
Germline variants of the immune checkpoint proteins PD‐1, PD‐l1 and CTLA‐4 and immune tolerance induction outcome in patients with inherited haemophilia A.
- Authors
Zuccherato, Luciana W.; Camelo, Ricardo M.; Chaves, Daniel G.; Rezende, Suely M.
- Abstract
Germline variants of the immune checkpoint proteins PD-1, PD-l1 and CTLA-4 and immune tolerance induction outcome in patients with inherited haemophilia A Intensive efforts in understanding the dynamics of the immune system related to ITI in PwHA have been made, and the PD-1/PD-L1 pathway might represent a paradigm change in this process. Haemophilia A (HA) represents one of the most common hereditary bleeding disorders in humans, caused by impaired production of functional factor VIII (FVIII) due to a pathogenic mutation in its coding gene ( I F8 i ).[1] Despite the effectiveness of HA treatment using well-established protocols based on FVIII replacement (plasma-derived or recombinant FVIII), approximately 30% of severe patients (residual FVIII activity < 1 IU/dL) develop neutralising alloantibodies (inhibitors) against the infused FVIII.
- Subjects
IMMUNE checkpoint proteins; PROGRAMMED cell death 1 receptors; IMMUNOLOGICAL tolerance; PROGRAMMED death-ligand 1; HEMOPHILIACS; AUTOIMMUNE diseases
- Publication
Haemophilia, 2023, Vol 29, Issue 5, p1366
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.14823