title: Non-genetic risk factors and their influence on the management of patients in the clinic.
abstract: The development of inhibitors is the most serious iatrogenic complication affecting patients with haemophilia. This complication is associated with impaired vital or functional prognosis, reduced quality of life and increased cost of treatment. The reasons why some patients develop antibodies to factor replacement and others do not remain unclear. It is however clear that inhibitor development results from a complex multifactorial interaction between genetic and non-genetic risk factors. Environmental influences implicated in increasing the risk of inhibitor formation can be viewed as modifiable risk factors. Therefore, identification of the non-genetic risk factors may offer the possibility of personalising haemophilia therapy by modifying treatment strategies in high-risk patients in the critical early phase of factor VIII exposure. In this article, we review the non-genetic factors reported as well as the potential impact of danger signals and the different scores for inhibitor development risk stratification.
authors.affiliation: Department of Haematology, La Paz University Hospital, Madrid, Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Álvarez
authors.forename: Immaculada
authors.initials: I
authors.lastname: Soto
authors.forename: Jan
authors.initials: J
authors.lastname: Astermark
mesh_terms: D000906:Antibodies / Q000097:blood*; D017281:Cost of Illness; D016207:Cytokines / Q000096:biosynthesis / Q000276:immunology; D019468:Disease Management; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy* / Q000191:economics / Q000276:immunology / Q000473:pathology; D006801:Humans; D011379:Prognosis; D011788:Quality of Life; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D016207:Cytokines; D005169:Factor VIII
keywords: environmental influences; haemophilia; inhibitors; risk factors