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Inhibitor development in hemophiliacs: The roles of genetic versus environmental factors

Lee, Christine A. ; Lillicrap, David and Astermark, Jan LU (2006) In Seminars in Thrombosis and Hemostasis 32. p.41561-41561
Abstract
Approximately 5 to 7% of patients with hemophilia A have inhibitory antibodies to factor (F) VIII, which increases to similar to 13% in patients with severe disease. The strongest determinant of the risk of inhibitor development identified is the type of mutation in the FVIII gene that gives rise to the disease. However, accumulating evidence clearly indicates that other genetic factors (e.g., major histocompatibility complex alleles and other immune-modulatory genes) and factors associated with treatment (e.g., type of FVIII concentrate, route of administration, and age of first exposure) may also influence the risk of inhibitor development. There is much interest in identifying such genetic and treatment-related factors to help minimize... (More)
Approximately 5 to 7% of patients with hemophilia A have inhibitory antibodies to factor (F) VIII, which increases to similar to 13% in patients with severe disease. The strongest determinant of the risk of inhibitor development identified is the type of mutation in the FVIII gene that gives rise to the disease. However, accumulating evidence clearly indicates that other genetic factors (e.g., major histocompatibility complex alleles and other immune-modulatory genes) and factors associated with treatment (e.g., type of FVIII concentrate, route of administration, and age of first exposure) may also influence the risk of inhibitor development. There is much interest in identifying such genetic and treatment-related factors to help minimize the risk of inhibitor development and improve treatment outcomes. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
inhibitory antibodies to FVIII, inhibitor development, FVIII prophylaxis
in
Seminars in Thrombosis and Hemostasis
volume
32
pages
41561 - 41561
publisher
Georg Thieme Verlag KG
external identifiers
  • wos:000238852400003
  • scopus:33745741765
  • pmid:16804830
ISSN
1098-9064
DOI
10.1055/s-2006-946909
language
English
LU publication?
yes
additional info
The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200)
id
e7aaca0e-3758-4ec5-aea9-7780fed65f8c (old id 686410)
date added to LUP
2016-04-01 16:43:58
date last changed
2020-01-12 19:40:53
@article{e7aaca0e-3758-4ec5-aea9-7780fed65f8c,
  abstract     = {Approximately 5 to 7% of patients with hemophilia A have inhibitory antibodies to factor (F) VIII, which increases to similar to 13% in patients with severe disease. The strongest determinant of the risk of inhibitor development identified is the type of mutation in the FVIII gene that gives rise to the disease. However, accumulating evidence clearly indicates that other genetic factors (e.g., major histocompatibility complex alleles and other immune-modulatory genes) and factors associated with treatment (e.g., type of FVIII concentrate, route of administration, and age of first exposure) may also influence the risk of inhibitor development. There is much interest in identifying such genetic and treatment-related factors to help minimize the risk of inhibitor development and improve treatment outcomes.},
  author       = {Lee, Christine A. and Lillicrap, David and Astermark, Jan},
  issn         = {1098-9064},
  language     = {eng},
  pages        = {41561--41561},
  publisher    = {Georg Thieme Verlag KG},
  series       = {Seminars in Thrombosis and Hemostasis},
  title        = {Inhibitor development in hemophiliacs: The roles of genetic versus environmental factors},
  url          = {http://dx.doi.org/10.1055/s-2006-946909},
  doi          = {10.1055/s-2006-946909},
  volume       = {32},
  year         = {2006},
}