CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Brief report Mortality in sickle cell patients on hydroxyurea therapy

The efficacy of hydroxyurea (HU) and its role in the reduction in mortality in sickle cell patients has been established. Never-theless, many patients still die of compli-cations 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 ane-mic, 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 repre-sent a subgroup of older patients, possi-bly with more severe disease and more severe organ damage. Such patients need early identification and prompt HU institu-tion. (Blood. 2005;105:545-547) © 2005 by The American Society of Hematolog