Symptoms

Haemolytic anaemia may occur with jaundice at birth, but usually appears later in life. The most frequent signs are:

• Anaemia which may range from mild to severe
• Jandice
• An enlarged spleen
• Leg ulcers

The jaundice is caused by the fact that a far greater than normal amount of haemoglobin is released into the bloodstream. This is converted in the liver to the bile pigment bilirubin, which is deposited in the skin and the whites of the eyes.

Causes

The average life of a red blood cell is 120 days. However, some people’s cells are more fragile and break up sooner, reducing the available haemoglobin. Spherocytosis is a condition in which the red cells, instead of being disc-shaped with a hollow on each surface (bi-concave discs), are shaped like little spheres (spherocytes). In this inherited disorder the cell fragility leads to rapid destruction and haemolytic anaemia, which is sometimes so severe that blood transfusion is needed.

Other causes of shortened red-cell life and haemolytic anaemia are:

• Sickle-cell disease
• The haemoglobinopathies
• Thalassaemia
• Malaria.

Haemolysis also occurs in other conditions, such as haemolytic disease of the newborn (rhesus disease), trauma, incompatible blood transfusions, deficiency of an enzyme called glucose-6-phosphate dehydrogenase (G-6PD), and a vitamin K overdose.

Treatment

In haemolytic anaemia, most of the breakdown of red cells occurs in the spleen. A marked and usually permanent improvement can be gained by removing the spleen. This is the standard treatment for all but the mildest cases.