Recent improvements in the clinical treatment of coagulation factor inhibitors.
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.
Franchini
Massimo
M
Immunohematology and Transfusion Center, University Hospital of Parma, Parma, Italy. mfranchini@ao.pr.it
Lippi
Giuseppe
G
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
D001323:Autoantibodies; D019774:Blood Coagulation Factor Inhibitors; D005169:Factor VIII; D005164:Factor IX