04/07/2011

Hemophilia At A Glance

Hemophilia is one of a group of inherited bleeding disorders that cause abnormal or exaggerated bleeding and poor blood clotting.

  • Hemophilia A and B are inherited in an X-linked recessive genetic pattern, so males are commonly affected while females are usually carriers of the disease.
     
  • Hemophilia A is caused by a deficiency of clotting Factor VIII, while hemophilia B (also called Christmas disease) results from a deficiency of Factor IX.
     
  • Hemophilia varies in its severity among affected individuals.
     
  • Symptoms include excessive bleeding from any site in the body; long-term damage to joints from repeated bleeding episodes is characteristic.
     
  • Treatment involves coagulation factor replacement therapy.
     
  • The formation of inhibitors to the treatment factor concentrates is a significant complication of treatment.
     
  • Gene therapy treatments are a source of active research and hold promise for the future.