Ivana Plameňová, Ján Staško
Haemophilia is a rare hereditary disorder caused by quantitative or qualitative deficiency of coagulation factor VIII (haemophilia A) or IX (haemophilia B). Its inheritance is X-linked and recessive. Haemophilia clinically presents with higher tendency to bleeding in males which correlates with the degree of coagulation factor deficiency. Repeated bleeding episodes into the locomotor apparatus lead to its permanent impairment and immobilization. The treatment of haemophilia is a substitution by factor VIII/IX concentrates; the dose depends on the degree of haemophilia, the extent, and the localization of bleeding or on the type of surgery. Appearance of FVIII/IX factor inhibitor is the most severe complication of haemophilia treatment; it results in non-efficacy of factor VIII/IX substitution and requires specific approach to the treatment. Centralized complex multidisciplinary care is important.