Neutrophil oxidative metabolism in sickle cell disease

Bacterial infections are the most frequent cause of death and an important factor of morbidity in sickle cell disease. A defect in oxidative metabolism of neutrophils from these patients has been reported as a possible cause for these infections. Since normal neutrophil functions are essential in the defense against bacteria, it seemed important to reassess the capacity of neutrophils from patients with sickle cell disease to undergo the metabolic events associated with phagocytic bactericidal activity. Accordingly, neutrophils from patients and controls were compared for their ability to reduce nitroblue tetrazolium dye, to activate the hexose monophosphate shunt, and to generate superoxide anion, hydrogen peroxide, and chemiluminescence. Patients' cells performed normally in each of these assays and, in addition, killed Staphylococcus aureus as well as did cells from controls. Thus, an abnormality of neutrophil oxidative metabolism cannot explain the propensity to bacterial infections in sickle cell disease.