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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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Submitted January 28, 2004
Accepted September 1, 2004
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*
Sickle Cell Center, Department of Medicine, Medical College of Georgia, Augusta, GA, USA
Office of Biostatistics & Bioinformatics, Medical College of Georgia, Augusta, GA, USA
* Corresponding author; email: akutlar{at}mcg.edu.
The efficacy of hydroxyurea (HU) and its role in the reduction in mortality in sickle cell patients has been established. However many patients still die from 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 cause of death (35%). The deceased and surviving patients did not differ significantly in average HU dose, baseline 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 BUN and creatinine. Sickle cell patients who die whilst on HU therapy may represent a subgroup of older patients, possibly with more severe disease and organ damage. Such patients need early identification and prompt HU institution.

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