INTRODUCTIONHemophilia A and B are X‐linked congenital bleeding disorders caused by mutations in clotting factor genes that result in deficiency of coagulation factor VIII (FVIII) and factor IX (FIX), respectively. The severity of bleeding is generally correlated with the clotting factor levels. The phenotype is severe when the clotting factor levels are lower than 1%, moderate when the levels are 1‐5%, and mild when the levels are 5‐40% of normal. Replacement of the deficient factor using specific factor concentrates, either plasma‐derived or recombinant, is a mainstay of therapy. Treatment consists of on‐demand regimens during bleeding episodes, either prophylactic treatment before invasive procedures or long‐term in patients with severe phenotype. Currently, the most serious and costly complication of factor replacement treatment is the development of antibodies, entitled inhibitors, to the infused factor. The presence of inhibitors typically makes factor replacement ineffective, and special treatment with bypassing agents such as activated prothrombin complex and activated factor VII concentrate is essential in managing bleeding events.Due to the improvement in the management of patients with hemophilia (PWH) A and B after the induction of factor concentrates the life expectancy of PWH has increased and is comparable to that among the non‐hemophilia population. Therefore,
Journal of Cardiac Surgery – Wiley
Published: Jan 1, 2018
Keywords: ; ; ; ; ;
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