title: Haemophilia B: current pharmacotherapy and future directions.
abstract: BACKGROUND
  Hemophilia B is a rare hereditary hemorrhagic disorder characterized by deficiency of the clotting factor IX (FIX). Hemophilia B patients experience mild to severe bleeding complications according to the degree of FIX defect. Nowadays, the most challenging complication of individuals with hemophilia B is the development of alloantibodies, which render the standard replacement therapy with FIX concentrates ineffective, exposing them to a significantly increased morbidity and mortality.
  
  
  METHODS
  This review summarizes the most important events leading to the development of the current FIX products available for the treatment of hemophilia B patients. In addition, it focuses on the more recent advances in the production of new FIX molecules aimed at improving the clinical management of such patients.
  
  
  CONCLUSIONS
  Although the availability of plasma-derived FIX concentrates has greatly improved the clinical management of hemophilia B patients, the introduction of FIX products using recombinant DNA technology has represented the most significant therapeutic progress in hemophilia B therapy, ensuring an advanced level of safety. The development of rFIX products with extended half lives will further improve the therapeutic armamentarium for hemophilia B patients.
authors.affiliation: Carlo Poma Hospital, Department of Transfusion Medicine and Hematology, Mantova, Italy. massimo.franchini@aopoma.it
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.forename: Francesco
authors.initials: F
authors.lastname: Frattini
authors.forename: Silvia
authors.initials: S
authors.lastname: Crestani
authors.forename: Carlo
authors.initials: C
authors.lastname: Bonfanti
mesh_terms: D005164:Factor IX / Q000627:therapeutic use*; D002836:Hemophilia B / Q000188:drug therapy* / Q000266:history; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D005164:Factor IX