Treatment of patients with haemophilia and inhibitory antibodies.
The development of inhibitory antibodies is a complication which arise in approximately 10% of patients with haemophilia A. The underlying genetic mutation is the single most important predisposing cause, although other risk factors have been identified. Periodic screening for inhibitors is a vital aspect of haemophilia care. The consequences of inhibitor development are very significant in terms of morbidity and cost. Several agents are now available for control of bleeding, but these are often very expensive. The most useful agents include recombinant activated factor VII, prothrombin complex concentrates and porcine factor VIII. It is possible to suppress antibody production with immune tolerance, which is successful in approximately 85% of cases and relapse is rare.
D019774:Blood Coagulation Factor Inhibitors / Q000097:blood / Q000276:immunology*; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005169:Factor VIII / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D006467:Hemophilia A / Q000097:blood / Q000235:genetics / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D007108:Immune Tolerance / Q000187:drug effects; D007223:Infant
D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates; D005169:Factor VIII; D015942:Factor VIIa