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April 2, 2003

Hydroxyurea and Sickle Cell Disease: A Chance for Every Patient

Author Affiliations

Author Affiliations: Division of Pediatric Emergency Medicine (Dr Weiner) and Department of Laboratory Medicine (Dr Brugnara), Children's Hospital Boston and Harvard Medical School, Boston, Mass (Drs Weiner and Brugnara).

JAMA. 2003;289(13):1692-1694. doi:10.1001/jama.289.13.1692

Basic research in hemoglobin polymerization over the last 50 years has shown that increasing fetal hemoglobin (HbF) levels significantly decreases sickle cell hemoglobin polymerization and erythrocyte sickling.1 Epidemiologic studies have shown that HbF concentration is the strongest determinant of clinical severity of sickle cell disease. Patients with low HbF concentrations have more frequent painful events and episodes of acute chest syndrome, as well as increased mortality, while those with higher concentrations of HbF have a milder disease course and longer lifespan.2-4 Increasing the levels of HbF has therefore been an important therapeutic target for the treatment of sickle cell disease.