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, Abdullah Kutlar

Research output: Contribution to journalArticlepeer-review

79 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)545-547
Number of pages3
Issue number2
StatePublished - Jan 15 2005

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology


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