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July 25, 1977

Iatrogenic β Thalassemia

JAMA. 1977;238(4):342. doi:10.1001/jama.1977.03280040062027

It is apparent that thalassemia has evolved into an American disease.1 With this in mind, Congress passed and the President signed the Cooley's Anemia Control Act in 1973, and thalassemia received special attention in the Genetics Act of 1976.

We wish to point out that the screening method relied on by most laboratories to detect thalassemia, the measurement of an increased A2 hemoglobin by cellulose acetate hemoglobin electrophoresis, is not accurate, and is frought with dangers of both false-positive and false-negative results. The method is good for the screening for sickle cell anemia, where at least 25% to 35% of the total hemoglobin is an abnormal hemoglobin S that migrates separately and distinctly from the normal hemoglobins A, F, and A2. The A2. hemoglobin content is small, normally less than 3% of the total hemoglobin. A substantial increase in A2 hemoglobin is usually between 3.5%