No abstract

Hydroxyurea is used for sickle-cell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes. Fetal hemoglobin, which is formed by γ-globin chains A and G, is present in a constant composition throughout fetal development: about 75% of G γ and 25% of A γ. In contrast, adult red cells contain about 40% of G γ and 60% of A γ. In the present study, we analyzed the effect of hydroxyurea induction on the γ chain composition of fetal hemoglobin in 31 sickle-cell disease patients treated with hydroxyurea. The control group was composed of 30 sickle-cell disease patients not treated with hydroxyurea in clinical steady state. The patients were older than 13 years and were not matched for age. All patients were seen at