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spherocytosis <haematology> A condition in which erythrocytes lose their biconcave shape and become spherical. It occurs as cells age and is also found in individuals with abnormal cytoskeletal proteins, (hereditary spherocytosis, a disorder that leads to haemolytic anaemia).
(18 Nov 1997)
spherocytosis, hereditary A familial congenital haemolytic anaemia characterised by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.
(12 Dec 1998)
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hereditary spherocytosis <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)
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