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

Christine A. Lee, David Lillicrap, Jan Astermark

Research output: Contribution to journalArticlepeer-review

39 Citations (SciVal)

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.
Original languageEnglish
Pages (from-to)41561
JournalSeminars in Thrombosis and Hemostasis
Volume32
DOIs
Publication statusPublished - 2006

Bibliographical note

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)

Subject classification (UKÄ)

  • Clinical Medicine

Keywords

  • inhibitory antibodies to FVIII
  • inhibitor development
  • FVIII prophylaxis

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