What is the primary role of hydroxyurea in the management of sickle cell disease?

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The primary role of hydroxyurea in the management of sickle cell disease is to increase fetal hemoglobin (HbF) production. Hydroxyurea acts as a fetal hemoglobin inducer, which helps to reduce the sickling of red blood cells. By increasing the levels of fetal hemoglobin, the drug reduces the amount of sickle hemoglobin (HbS) and thus decreases the frequency and severity of painful vaso-occlusive crises, which are a hallmark of sickle cell disease.

Higher levels of fetal hemoglobin lead to a reduced polymerization of sickle hemoglobin under low oxygen conditions, making the red blood cells less likely to sickle. This effect contributes significantly to the overall management of sickle cell disease, improving patient outcomes and quality of life.

Other options like increasing white blood cell count, lowering blood pressure, or enhancing iron absorption do not accurately reflect the primary action or therapeutic goal of hydroxyurea in this context. The focus is on the management of the erythrocyte abnormalities characteristic of sickle cell disease, where increasing fetal hemoglobin serves as a pivotal intervention.

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