MEDICINE : THALASSEMIA(Cooley’s anemia, Mediterranean anemia, erythroblastic anemia)

• School: Kerala University of Health Sciences - KUHS
• Department: Psychology
• Course: MEDICINE

Document Summary

Thalassemic syndromes are disorders of hemoglobin synthesis with decreased production of either alpha or beta polypeptide chains of hemoglobin molecules. Features first described by thomas b cooley in 1925 are seen primarily in mediterranean populations. The disease is termed thalassemia when there is deficient synthesis of the -chain and -thalassemia when the -chain is deficient. In -thalassemia there is an excess of -chains, producing unstable hemoglobins" that damage the erythrocytes and increase their vulnerability to destruction. In heterozygotes, the disease is mild and is called thalassemia minor or thalassemia trait. The presence of such intracellular inclusion bodies (fessas bodies) leads to increased erythrocyte hemolysis and severe ineffective hematopoiesis. Two other forms of thalassemia major that represent -thalassemia also exist. Hemoglobin h disease, which is a very mild form of the disease in which the patient may live a relatively normal life. Hemoglobin bart"s disease, with hydrops fetalis, in which the infants are stillborn or die shortly after birth.