Pavol Hollý, Peter Kubisz, Ján Staško
Factor VIII (FVIII) inhibitors represent a serious complication of substitution therapy in congenital hemophilia A, affecting
5-7 % patients. Inhibitors render the standard substitution therapy with FVIII ineffective and increase the risk of
bleeding and subsequent complications. Immune tolerance induction (ITI) is the only proven therapeutic approach for
inhibitor eradication. It involves a long-term regular administration of FVIII alone or in combination with immunomodulatory/
immunosuppressive agents or techniques. Its success rate in severe hemophilia A is 70 – 80 %.The prevention
and treatment of haemorrhage, usually with bypassing agents, is an inevitable part of ITI. Most of the clinical experience
with ITI is derived from persons with severe hemophilia A; the data are limited for moderate and mild hemophilia.