Acquired hemophilia (AH) is a rare autoimmune disorder recognized by bleeding that occurs in patients with a personal and family history negative for hemorrhages. Autoimmune disorders occur when the body’s immune system mistakenly attacks healthy cells or tissue. Acquired hemophilia shares some similarities with congenital hemophilia but is a distinctly different condition. Normally, blood clots after injury or surgical procedures by a complicated process involving a number of different proteins. These are mostly made in the liver and then released into the blood.
In acquired hemophilia, the person may not have a history of bleeding. At some point in their life their immune system develops an antibody to their own factor VIII – this antibody stops FVIII working normally and removes it from the circulation. This lack of factor VIII activity is why these patients bleed; sometimes this bleeding is in response to trauma, and sometimes patients will bleed for no apparent reason.
In about half the cases of acquired hemophilia that are diagnosed, the cause is never established. Part of the immune system becomes over-active towards Factor VIII.
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