The modern treatment of haemophilia: a narrative review

摘要: Haemophilia A and B are X chromosome-linked bleeding disorders that included among the rare diseases caused by mutations in genes for factor VIII (FVIII) IX (FIX)1. Both these clotting factors part of intrinsic pathway blood coagulation. Individual with haemophilia may have severe, moderate or mild forms diseases, defined plasma levels 1% less, 2 to 5% 6 30%, respectively. The prevalence is 1 case 5,000 male live births, while 30,0002,3. Although patients usually bleed excessively only after trauma surgery, those severe experience frequent episodes spontaneous excessive bleeding, particularly into joints muscles, minor trauma3. modern management began 1970s currently includes several plasma-derived recombinant products4. This article reviews recent history, current knowledge, most important progress expected improvements care.