title: Inhibitors of propagation of coagulation (factors VIII, IX and XI): a review of current therapeutic practice.
abstract: The management of patients with congenital haemophilia who develop alloantibodies against factors of the propagation phase of blood coagulation, commonly known as inhibitors, is the most important challenge facing haemophilia caregivers at present, as this complication not only compromises the efficacy of replacement therapy but also consumes an enormous amount of economic resources. Development of inhibitors further complicates the clinical course of severe haemophilia, with a prevalence of up to 30% in patients with haemophilia A (factor VIII deficiency) and up to 5% in those with haemophilia B (factor IX deficiency) and haemophilia C (factor XI deficiency). While the short-term goal of treatment of patients who develop alloantibodies is the control of bleeding, the eradication of the inhibitor is the main long-term goal. The management of severe bleeding episodes and the eradication of the autoantibody are also the mainstays of treatment of patients with acquired haemophilia, a rare but life-threatening haemorrhagic condition characterized by the development of inhibitory autoantibodies against coagulation factor VIII. The most recent options available for treating patients with congenital haemophilia complicated by inhibitors and acquired haemophilia because of autoantibodies against factor VIII are summarized in this review article.
authors.affiliation: Immunohaematology and Transfusion Centre, Department of Pathology and Laboratory Medicine, University Hospital of Parma, Parma, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.forename: Pier Mannuccio
authors.initials: PM
authors.lastname: Mannucci
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D019774:Blood Coagulation Factor Inhibitors / Q000627:therapeutic use; D005164:Factor IX / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D005172:Factor XI / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy; D002836:Hemophilia B / Q000188:drug therapy; D006801:Humans
chemical_list: D000925:Anticoagulants; D019774:Blood Coagulation Factor Inhibitors; D005169:Factor VIII; D005164:Factor IX; D005172:Factor XI