title: Pharmacotherapy of haemophilia A.
abstract: BACKGROUND
  Haemophilia A is due to factor VIII (FVIII) deficiency. The main treatment is replacement therapy with FVIII concentrates. However, these concentrates carried a high risk of blood-borne viral infections and still have a high risk of inducing anti-FVIII inhibitors.
  
  
  METHODS
  An overview of products available and therapeutic options for haemophilia A management in order to help in decision making. A literature search using Medline with the keywords: 'haemophilia', 'factor VIII', 'therapy', 'inhibitor', 'concentrate', 'bleeding', 'prophylaxis', 'on demand', 'plasma-derived', 'recombinant', 'coagulation factors', 'immunotolerance' was performed. The years 1960 - 2010 are included.
  
  
  CONCLUSIONS
  Progress in management of patients with haemophilia A has allowed increased life expectancy and quality of life. There is evidence that prophylaxis prevents or, at least, slows down arthropathy development when started early in childhood. FVIII concentrates have achieved high levels of blood-borne pathogen safety. However, treatment is frequently complicated by development of FVIII-neutralizing inhibitors, which prevent control of bleeding and predispose to a high morbidity and mortality risk. Bypassing agents are effective in bleeding treatment in a high percentage of cases. Prophylaxis with bypassing agents and their use in combination are offering opportunities in management of inhibitor patients. More evidence is necessary to understand how to prevent and manage this complication.
authors.affiliation: Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Milan, Italy. alessandro.gringeri@unimi.it
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gringeri
authors.forename: Myrvet
authors.initials: M
authors.lastname: Muça-Perja
authors.forename: Lucia
authors.initials: L
authors.lastname: Mangiafico
authors.forename: Sylvia
authors.initials: S
authors.lastname: von Mackensen
mesh_terms: D000818:Animals; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans
chemical_list: D001779:Blood Coagulation Factors