sickle cell anaemia


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sickle cell anaemia

An inherited blood disease mainly affecting black people and those of Mediterranean origin. The basic defect is in the HAEMOGLOBIN molecule of the red blood cells, which is abnormal (haemoglobin S) and deforms in conditions of low oxygen tension causing the red cells to become sickle-shaped (sickling) and to rupture readily. The HETEROZYGOUS form is comparatively mild; the HOMOZYGOUS form is severe and dangerous and an incidental illness can bring on a sickling crisis calling for prompt and energetic treatment.