TY - JOUR
T1 - Mortality in sickle cell patients on hydroxyurea therapy
AU - Bakanay, Sule M.
AU - Dainer, Erin
AU - Clair, Betsy
AU - Adekile, Adekunle
AU - Daitch, Lisa
AU - Wells, Leigh
AU - Holley, Leslie
AU - Smith, David
AU - Kutlar, Abdullah
PY - 2005/1/15
Y1 - 2005/1/15
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=11244348903&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=11244348903&partnerID=8YFLogxK
U2 - 10.1182/blood-2004-01-0322
DO - 10.1182/blood-2004-01-0322
M3 - Article
C2 - 15454485
AN - SCOPUS:11244348903
SN - 0006-4971
VL - 105
SP - 545
EP - 547
JO - Blood
JF - Blood
IS - 2
ER -