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Blood, 15 January 2005, Vol. 105, No. 2, pp. 545-547.
Prepublished online as a Blood First Edition Paper on September 28, 2004; DOI 10.1182/blood-2004-01-0322.


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CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Brief report

Mortality in sickle cell patients on hydroxyurea therapy

Sule M. Bakanay, Erin Dainer, Betsy Clair, Adekunle Adekile, Lisa Daitch, Leigh Wells, Leslie Holley, David Smith, and Abdullah Kutlar

From the Sickle Cell Center, Department of Medicine, Office of Biostatistics and Bioinformatics, Medical College of Georgia, Augusta.

The efficacy of hydroxyurea (HU) and its role in the reduction in mortality in sickle cell patients has been established. Nevertheless, many patients still die of complications of this disease while on HU. Of the 226 patients treated with HU at our center, 38 died (34 of sickle cell–related causes). Acute chest syndrome (ACS) was the most common (35%) cause of death. Deceased and surviving patients did not differ significantly in average HU dose, baseline fetal hemoglobin (Hb F), or maximum Hb F response. However, the deceased patients were significantly older when HU was instituted, were more anemic, and more likely to have BAN or CAM haplotypes. They also had significantly higher serum blood-urea-nitrogen (BUN) and creatinine levels. Sickle cell patients who die while on HU therapy may represent a subgroup of older patients, possibly with more severe disease and more severe organ damage. Such patients need early identification and prompt HU institution.


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