thalassaemia

(redirected from Cooleys anaemia)
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thal·as·se·mi·a

, thalassanemia (thal'ă-sē'mē-ă, -ă-să-nē'mē-ă)
Any of a group of inherited disorders of hemoglobin metabolism in which there is impaired synthesis of one or more of the polypeptide chains of globin; several genetic types exist, and the corresponding clinical picture may vary from barely detectable hematologic abnormality to severe and fatal anemia. People of Mediterranean, extraction are more often affected than others by this type of anemia.
Synonym(s): thalassaemia, thalassanaemia.
[G. thalassa, the sea, + haima, blood]

thalassaemia

One of several hereditary abnormalities of synthesis of the globin chains of HAEMOGLOBIN leading to severe ANAEMIA. The disorder is common in the area surrounding the Mediterranean sea. When the abnormality is HETEROZYGOUS (thalassaemia minor), disability is minimal, but when HOMOZYGOUS (thalassaemia major), anaemia may be severe with breathlessness, jaundice, spleen enlargement and sometimes physical and mental retardation. Treatment is by blood transfusion and bone marrow transplantation. From the Greek words thelazia , the sea, and haima , blood.

thalassaemia

inherited disorders of haemoglobin metabolism; of variable presentation but more common amongst people from the Eastern Mediterranean; erythrocytes tend to clump especially in conditions of low oxygen tension (e.g. high altitude; tissues distal to a tourniquet)
  • thalassaemia major Cooley's anaemia; thalassaemia presenting in infancy or early childhood in homozygous individuals

  • thalassaemia minor asymptomatic presentation in heterozygous individuals

thal·as·se·mi·a

, thalassanemia (thal'ă-sē'mē-ă, -ă-să-nē'mē-ă)
Any of a group of inherited disorders of hemoglobin metabolism with impaired synthesis of one or more polypeptide chains of globin; several genetic types exist.
Synonym(s): thalassaemia, thalassanaemia.
[G. thalassa, the sea, + haima, blood]