title: Recent improvements in the clinical treatment of coagulation factor inhibitors.
abstract: The mainstay of therapy in patients with congenital hemophilia is factor replacement. However, the development of inhibitors in these patients is a major complication that represents an important challenge in hemophilia care. Development of inhibitors complicates the clinical course of severe hemophilia in up to 30% of patients with hemophilia A and up to 5% of patients with hemophilia B. Although the main short-term objective of the treatment of alloantibodies against factors VIIII and IX is to control the bleeding diathesis, the eradication of the inhibitor is the leading long-term goal. The management of severe bleeding episodes and the definitive eradication of the autoantibody are also the two main options of the clinical management of patients with acquired hemophilia, a rare but life-threatening hemorrhagic condition. The most recent options available for treating patients with acquired hemophilia and congenital hemophilia with inhibitors are addressed in this review.
authors.affiliation: Immunohematology and Transfusion Center, University Hospital of Parma, Parma, Italy. mfranchini@ao.pr.it
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lippi
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D019774:Blood Coagulation Factor Inhibitors / Q000032:analysis / Q000037:antagonists & inhibitors*; D005164:Factor IX / Q000037:antagonists & inhibitors; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology* / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D006801:Humans
chemical_list: D001323:Autoantibodies; D019774:Blood Coagulation Factor Inhibitors; D005169:Factor VIII; D005164:Factor IX