Product type and other environmental risk factors for inhibitor development in severe hemophilia A.

ABSTRACT: The development of FVIII inhibitory antibodies is currently the most challenging complication of treatment, affecting ~30% of severe hemophilia A patients. These inhibitors inactivate FVIII, rendering the treatment ineffective, causing disability and increasing morbidity and mortality. Inhibitor development results from a complex multicausal immune response involving both genetic and environmental risk factors. One of the most important modifiable risk factors is the source of FVIII products, eg, plasma-derived or recombinant FVIII. Other environmental risk factors, such as age at first treatment, regimen, and intensity of treatment, could contribute to inhibitor development. Severe bleeds, surgery, concomitant infections, or vaccinations may all be events initiating danger signaling resul

SUBMITTER: Peyvandi F 

PROVIDER: S-EPMC6055565 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature