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Hereditary Spherocytosis (HS)

Hereditary spherocytosis (HS) is an inherited blood condition which is lifelong. There is a change in one of the proteins making up the membrane of all the red cells, allowing the red cells to change from their usual disc shape into a sphere (“spherocyte”). These spherocytes are more prone to being damaged as they travel in the circulation than disc shaped cells, and they tend to break up more easily, so the average lifespan of an individual red cell in the circulation is shortened and the patient has a mild chronic anaemia.

As the cells break up, the red pigment (haemoglobin) is released and this is then broken down further by the body, leading to a yellowish tinge (jaundice) seen most easily in the whites of the eyes. In patients with spherocytosis, this increased breakdown of red cells (haemolysis) is accentuated even more during times of other intercurrent illness (e.g., colds, ‘flu), so the jaundice can worsen during these times and the anaemia can worsen a little.

There is usually good recovery to the steady state as the bone marrow compensates by producing more red cells, and this process is helped by taking a simple vitamin, folic acid.

Occasionally an intercurrent infection can occur which temporarily prevents the bone marrow from providing adequate replacement red cells and, in this situation, the anaemia can become quite severe, even to the point of needing a blood transfusion. The most common infection to cause this is a virus known as parvovirus B19, which is one of the common childhood viral illnesses. Once a person has been exposed to parvo B19, they make antibodies so the infection virtually never returns.

Patients with HS have an increased tendency to create gallstones (about 50% will have them by age 50) but this does not always mean that a gallstone operation is needed.

Some patients have more severe episodes of anaemia than others. If problems keep recurring, even to the point of needing repeated blood transfusions, an operation to remove the spleen (splenectomy) might be considered. This is almost always successful in controlling the severity of the anaemia, although the red cells themselves remain abnormal. However, splenectomy is a major operation and is not without risks, so this option is always considered very carefully for each individual and generally is far less commonly performed than decades ago. 

Contact us

If you have any queries relating to this information, please contact the Haematology (blood) service.

About this information

Service:
Haematology (blood)

Reference:
R/014

Approval date:
19 May 2021

Review date:
1 May 2026

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This page provides general information only. It is developed by clinical staff and is reviewed regularly every 3 years for accuracy. For personal advice about your health, or if you have any concerns, please speak to your doctor.