This Article  • Full text  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (8)  • Contact me when this article is cited  Related Content  • Related letter  • Related article  • Similar articles in JAMA  Social Bookmarking   What's this?

A Chance for Every Patient

Debra L. Weiner, MD, PhD; Carlo Brugnara, MD

JAMA. 2003;289:1692-1694.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

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.

In 1984 Platt et al⁵ reported that hydroxyurea, a cytostatic, myelosuppressive agent used for over 30 years as a chemotherapeutic drug to treat cancer, could increase levels of HbF in patients with sickle cell disease. Several uncontrolled clinical studies followed, demonstrating improvements . . . [Full Text of this Article]

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).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED LETTER

Long-term Use of Hydroxyurea for Sickle Cell Anemia
Lawrence Feldman, Sandra Allen, Maxwell Westerman, Lori Feldman, Alice Gilman-Sachs, and Kenneth Beaman
JAMA. 2003;290(6):752-753.
EXTRACT | FULL TEXT  

RELATED ARTICLE

Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia: Risks and Benefits Up to 9 Years of Treatment
Martin H. Steinberg, Franca Barton, Oswaldo Castro, Charles H. Pegelow, Samir K. Ballas, Abdullah Kutlar, Eugene Orringer, Rita Bellevue, Nancy Olivieri, James Eckman, Mala Varma, Gloria Ramirez, Brian Adler, Wally Smith, Timothy Carlos, Kenneth Ataga, Laura DeCastro, Carolyn Bigelow, Yogen Saunthararajah, Margaret Telfer, Elliott Vichinsky, Susan Claster, Susan Shurin, Kenneth Bridges, Myron Waclawiw, Duane Bonds, and Michael Terrin
JAMA. 2003;289(13):1645-1651.
ABSTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Blood mononuclear cell gene expression profiles characterize the oxidant, hemolytic, and inflammatory stress of sickle cell disease
Jison et al.
Blood 2004;104:270-280.
ABSTRACT | FULL TEXT  

Long-term Use of Hydroxyurea for Sickle Cell Anemia
Feldman et al.
JAMA 2003;290:752-753.
FULL TEXT  

Hydroxyurea for Sickle Cell Anemia Might Lower Mortality
JWatch Pediatrics 2003;2003:14-14.
FULL TEXT  

Hydroxyurea for Sickle Cell Anemia Might Lower Mortality
JWatch General 2003;2003:5-5.
FULL TEXT