Which of the following is the primary mechanism for anemia in the thalassemias?
a. An imbalance in the rate of globin chain synthesis
b. Impaired iron incorporation into the protoporphyrin ring
c. Erythroid hypoplasia in the bone marrow
d. Abnormal amino acid structure of a globin chain
ANS: A
An imbalance exists in the rate of globin chain synthesis in the thalassemias, and this is the primary mechanism leading to anemia. A build-up of the globin chain is produced in excess, and this causes damage to the red cell. Erythroid hyperplasia is present in the bone marrow (ineffective erythropoiesis).
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