thalassaemia

(Cooley's anaemia)

n.

a hereditary blood disease, widespread in the Mediterranean countries, Asia, and Africa, in which there is an abnormality in the protein part of the haemoglobin molecule. The affected red cells cannot function normally, leading to anaemia. Other symptoms include enlargement of the spleen and abnormalities of the bone marrow. Individuals inheriting the disease from both parents are severely affected (thalassaemia major), but those inheriting it from only one parent are usually symptom-free. Patients with the major disease are treated with repeated blood transfusions or bone marrow transplantation. The disease can be detected by prenatal diagnosis.