Abstract
Congenital hemoglobin mutations may alter the delicate balance of free-radical generation and antioxidant defense systems in the red cell. Oxidative stress may thus play a role in the pathophysiology of the clinical manifestations of the disease. We assessed the total antioxidant status in steady-state sickle cell anemia (SCA) patients and related it to certain hematological parameters and their recent clinical history. Forty (25 males/15 females) adult SCA patients and 30 age-matched controls were studied. All patients and control subjects had total antioxidant status (TAS), hematocrit, white blood cells, platelets and reticulocyte count done. The results showed that TAS levels were about 50% lower in the SCA patients compared with the controls. Among the SCA patients, 57.1% of those with TAS levels <1.00 mmol/L had bone pain crisis >3 times in the past year, compared with 16% in those with TAS levels >1.00 mmol/L. Total leukocyte count and platelets were also significantly higher in the SCA patients than controls. Our data support the growing evidence that oxidative stress has a role to play in the pathophysiology of SCA and intervention aimed at increasing the antioxidant capacity of these patients may be beneficial.
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