What is acquired haemophilia?
Bleeding disorders are a group of conditions of the blood clotting (coagulation) system in which bleeding is prolonged and at times excessive. Acquired haemophilia is a bleeding disorder that shares similarities with congenital haemophilia, but it’s not a condition you are born with. Unlike haemophilia, the production of factor V111 is destroyed as it is made, this condition is normally associated with the aging process, and it can occur at any age. Both males and females can be equally affected by this condition.
What causes acquired haemophilia?
The cause of acquired haemophilia is not always established, although it is linked with the immune system not working properly. It can be associated with other illnesses such as rheumatoid arthritis, ulcerative colitis, asthma and psoriasis; in addition, it can be triggered by a reaction to some drugs during pregnancy.
The immune system consists of a number of blood cells, lymph glands and the spleen. Its function within our bodily systems is, to protect them from attacks from viruses, bacteria and other proteins that the body considers as unrecognisable to the system. Acquired haemophilia is caused by the immune system developing an antibody which attacks and destroys factor V111 (the proteins that are responsible for assisting with coagulation). The terminology for this antibody is ‘Inhibitor’; this is because it inhibits the action of factor V111.
How many people are affected by acquired haemophilia?
Acquired haemophilia affects one person in every million, therefore it is extremely rare and its not common for a GP to have experience of this condition.
What are the signs and symptoms of acquired haemophilia?
The signs of acquired haemophilia can be extensive bruising, bleeding into muscles which can be very painful, prolonged bleeding following surgery, blood in the urine and/or from the bowels.
Acquired haemophilia is diagnosed through a blood test, this is usually undertaken in a hospital laboratory which is attached to a haemophilia centre.
How is acquired haemophilia treated?
Treatments are available and usually very successful. Several types of clotting factors are available to stop bleeding, blood plasma, recombinant products and desmopressin if the bleeding is not too severe. The antibody can be eradicated by using drugs such as, steroids, cytotoxic drugs, and sometimes Rituximab.
Will I get rid of acquired haemophilia?
Eradicating the inhibitor is possible. Different routes of transmission are available, you can discuss these different options with your healthcare team.
Tablets can be taken over a course of many weeks.
A drip containing a course of immunoglobulins (IVIg) can be administered over a period of 2 – 5 days.
Occasionally a process which involves passing the blood through a machine is used to try to filter out the inhibitor. This is known as plasmapheresis.
Risks and limitations
- Always contact your haemophilia centre immediately if any signs of bleeding or bruising occur. If you are worried about anything, your centre staff will be there to advise you.
- Avoid activities which may put you at risk or injury
- Don’t take aspirin or non steroidal anti inflammatory drugs such as ibuprofen.
- Don’t take any drugs not prescribed to you by your haemophilia centre without speaking to them first
- Don’t have injections into your muscles if your inhibitor has not been eradicated.
If in doubt ask your nurse of haemophilia consultant
