hereditary spherocytosismedical dictionary

<haematology> A hereditary disorder that leads to a chronic haemolytic anaemia due to an abnormality in the red blood cell membrane.

This disorder is caused by a defective gene. Red cells are resistant to stress and rupture easily. Infants may appear jaundiced and pale. Fatigue, weakness and shortness of breath are other symptoms that may be seen in older patients. The spleen may also be enlarged.

Treatment includes splenectomy (removal of the spleen). After this is accomplished the life-span of the red blood cells returns to normal.

(27 Sep 1997)