Sideroblastic anaemia

Anaemia is a condition where the number of red blood cells or the amount of haemoglobin in red blood cells is less than normal. Sideroblastic anaemia is a disorder where the body produces enough iron but is unable to put it into the haemoglobin.

Red blood cells contain haemoglobin. This is the substance that makes blood red. Its main purpose is to carry oxygen around the body. If the body does not receive enough oxygen, various symptoms occur. These include tiredness, weakness and lack of energy. If the anaemia becomes more severe, it can become life threatening.

Anaemia can be caused by many things, including losing a lot of blood after surgery or an accident, a decrease in the number of red cells being produced in the bone marrow or an increase in the number of red cells being destroyed by the body.

What is sideroblastic anaemia?

Sideroblastic anaemia is a disorder where the body produces enough iron but is unable to put it into the haemoglobin. A sideroblast is a cell in which there is an abnormally large build up or iron.

How common is it?

Again, sideroblastic anaemia is a rare disorder.

Is it inherited?

Some forms of the condition are inherited as an X-linked trait. Some forms, usually seen in adults, are acquired by exposure to alcohol, lead or drugs. Others are not inherited but appear for no reason (idiopathic) but these tend to affect older people.

What are the symptoms?

Symptoms include weakness, fatigue and difficulty in breathing. The build up of iron in the body can cause an enlarged spleen or liver, and abnormal heart rhythms. The symptoms tend to be mild in childhood but become more severe in adulthood.

How is it treated?

There are various drugs that are used to treat sideroblastic anaemia, including pyridoxine. Iron overload can be treated by another drug, deferoxamine. Blood transfusions are also used to treat the condition.

Compiled by:
The Haematology department in collaboration with the Child and Family Information Group.
Last review date:
April 2007
Ref:
F070211