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).
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.
Weiner DL, Brugnara C. Hydroxyurea and Sickle Cell Disease: A Chance for Every Patient. JAMA. 2003;289(13):1692–1694. doi:10.1001/jama.289.13.1692
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