Abstract
The development of inhibitor antibodies is perhaps the most serious complication of coagulation factor replacement therapy. A complex interaction of several variables leads to inhibitor formation in congenital haemophilia, while acquired haemophilia represents a failure of the immune tolerance mechanisms that regulate a normal immune response to factor VIII (FVIII). The immune response to FVIII is dependent upon the interaction of different CD4+ T-cell subsets (Th1, Th2 and Th3) specific for FVIII. Failure to activate regulatory CD4+ cells likely plays a crucial role in the development of FVIII inhibitors. Although the basic mechanisms of the immune response to FVIII in the setting of factor replacement therapy are being elucidated, a clear understanding of the relevance of these mechanisms in the context of successful immune tolerance therapy and ultimately gene therapy, awaits further study.
Original language | English (US) |
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Pages (from-to) | 30-36 |
Number of pages | 7 |
Journal | Haemophilia |
Volume | 12 |
Issue number | SUPPL. 6 |
DOIs | |
State | Published - Dec 2006 |
Keywords
- CD4+ T cells
- Factor IX
- Factor VIII
- Haemophilia
- Immune response
- Inhibitors