Prevention and prediction of inhibitor risk.

摘要: Summary: Studies of determinants the development inhibitory antibodies in patients with haemophilia indicate that this is a complex process involving several factors. The foundation characterized by T- and B-cell repertoire antigen presenting cells to elicit an immune response deficient factor, pre-disposing needed. Hence, absence certain set circumstances, there will be no risk for inhibitors. Conversely, fundamentally at risk, genetic non-genetic factors might add risk. These may additive or interactive, ultimately promote counteract reaction modifying regulators cytokine profile individual. In some subjects, only minor inflammatory signals needed, whereas others more pronounced pro-inflammatory state required. Regarding markers other than type mutation HLA class II molecules, polymorphisms various regulatory genes have been associated inhibitor associations not, however, consistent across all patient groups. reason not clear, but could related study design statistical power, family relationships among those studied, complexity interacting molecules ethnic genomic variation. Hemophilia Inhibitor Genetics Study (HIGS) has identified additional candidates within intracellular pathways, which require evaluation fully appreciated. case factors, overall view system challenges HIGS data suggest it possible calculate score identify high before start treatment. By doing so, hopefully future prevent formation inhibitors these offering therapeutic options native factor VIII IX molecule setting. (Less)