Mortality in Sickle Cell Patients on Hydroxyurea Therapy

Investigators from the Medical College of Georgia, Augusta, performed a retrospective analysis of 226 patients with sickle cell disease (SCD) aged 16 to 68 years of age who were treated with hydroxyurea (HU) over a 15-year period. The investigators compared the characteristics of the 38 patients who died during this period with those of surviving patients. Of these 38 patients, 26 were on HU therapy at the time of death, and 12 were not compliant with therapy. Four patients died of causes unrelated to SCD. Of the 34 patients who died of sickle cell–related causes, 12 died of acute chest syndrome (ACS), 5 of multiorgan failure, 4 of stroke, 2 of end-stage renal disease, and 1 each of cardiac arrest, sepsis, cardiac arrhythmia, and pulmonary embolism. In 7 patients, the precise cause of death could not be determined. Deceased and surviving patients did not differ significantly with regard to average HU dose, duration of HU therapy, baseline hemoglobin F (Hb F), or maximum Hb F response. Men and women had equivalent survival rates.

The deceased group of patients was older (30.6 …