Hereditary and acquired hemophilia

Danas su poznate tri vrste nasljedne hemofilije : najčešća hemofilija A, zatim hemofilija B, te vrlo rijetka hemofilija C. Ostalih 5 do 10% nasljednih koagulacijskih poremećaja uzrokuju deficiti drugih faktora koji uključuju faktor II, V,VII, X, XII i fibrinogen i nasljeđuju se autosomno recesivno. Stečena hemofilija označava rijetko stanje karakterizirano stvaranjem i prisutnošću antitijela na faktore zgrušavanja, najčešće na faktor VIII. Liječenje hemofilije ovisi o vrsti i težini bolesti, a temelji se na nadomjesnoj terapiji nedostatnog čimbenika zgrušavanja/ faktora VIII u hemofiliji A, faktora IX u hemofiliji B, odnosno faktora XI u vrlo rijetkoj hemofiliji C/. Terapija stečene hemofilije temelji se na dva principa : zaustavljanju krvarenja i odstranjenju /eliminaciji/ inhibitora. Genska terapija za nasljednu hemofiliju ovoga se trenutka također istražuje u kliničkim studijama. Obzirom da je hemofilija nasljedna bolest u čijoj je patofiziologiji osnovna mutacija gena logičan je razvoj terapije u tom smjeru. Međutim, ta se vrsta terapije za sada još ne primjenjuje u rutinskom liječenju.Today, three types of hereditary hemophilia are known: the most common hemophilia A, then hemophilia B, and the very rare hemophilia C. The other 5 to 10% of hereditary coagulation disorders are caused by deficits of other factors, which include factor II, V, VII, X, XII and fibrinogen and inherit is autosomal recessive. Acquired hemophilia means a rare condition characterized by the formation and presence of antibodies to clotting factors, most often to factor VIII. Treatment of hemophilia depends on the type and severity of the disease, and is based on the replacement therapy of the insufficient clotting factor/factor VIII in hemophilia A, factor IX in hemophilia B, or factor XI in the very rare hemophilia C/. The therapy of acquired hemophilia is based on two principles: stopping the bleeding and removing the inhibitor..Gene therapy for hereditary hemophilia is also currently being investigated in clinical trials. Considering that hemophilia is a hereditary disease in the pathophysiology of which the basic gene mutation is the logical development of therapy in this direction. However, this type of therapy is not yet used in routine treatment