Pathogenic immune response to therapeutic factor VIII: exacerbated response or failed induction of tolerance?

ABSTRACT: Therapeutic factor VIII is highly immunogenic. Despite intensive research in the last decades, the reasons why 5-30% of patients with hemophilia A (of all severities) develop inhibitory anti-factor VIII antibodies (inhibitors) following replacement therapy remain an enigma. Under physiological conditions, endogenous factor VIII is recognized by the immune system. Likewise, numerous observations indicate that, in hemophilia A patients without inhibitors, exogenous therapeutic factor VIII is immunologically assessed and tolerated. A large part of the research on the immunogenicity of therapeutic factor VIII is attempting to identify the 'danger signals' that act as adjuvants to the deleterious anti-factor VIII immune responses. However, several of the inflammatory assaults concomitant to fac

SUBMITTER: Varthaman A 

PROVIDER: S-EPMC6355482 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature