Haemophilia

What is congenital haemophilia?
Haemophilia is an inherited (congenital) bleeding disorder that occurs frequently in males. It occurs when the levels of certain clotting factors (factors VIII or IX) are reduced or are completely absent. This is because the gene that controls the production of the clotting factor does not work properly. In the case of haemophilia A, there is a problem with factor VIII and in haemophilia B it is factor IX. These clotting factors are essential in order to prevent uncontrolled bleeding. Without these clotting factors, bleeding will continue until treatment is given because the body is not able to produce sufficient natural clotting factors to form a clot to stop it.

What are inhibitors?
The normal treatment for haemophilia is to replace the missing clotting factor. However, some people’s immune systems respond to these replacement factors by developing antibodies known as inhibitors that destroy the clotting factor.

What is acquired haemophilia?
Acquired haemophilia is not inherited, and cannot be passed on in the family. It may develop suddenly when a person’s immune system starts to destroy his/ her own clotting factors by mistake. This reduction in the amount of clotting factor prevents the blood from clotting naturally, so bleeding continues

Who gets acquired haemophilia?
Acquired haemophilia is very rare, occurring in about 1-4 per million people every year. Men and women are equally likely to be affected. It is usually seen in older people, but has been associated with a wide variety of conditions, such as rheumatoid arthritis, pregnancy and as a reaction to certain drugs.