Increased In Vivo Activation of Neutrophils and Complement in Sickle Cell Disease

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  • Department of Clinical Chemistry, and Department of Clinical Immunology and Transfusion Medicine, University Hospital, Department of Paediatrics and Child Health, Faculty of Medicine, University of Khartoum, Uppsala, Sweden

Eight patients with homozygous sickle cell anemia, 15 heterozygotes, and eight control individuals were investigated with respect to plasma concentrations of the inflammatory markers lysozyme and myeloperoxidase and the complement activation marker C3d. The patients showed significantly increased levels of myeloperoxidase and C3d, but not lysozyme, compared with the heterozygotes and the controls. The heterozygotes were also significantly different from the controls with regard to C3d concentration. The concentrations of myeloperoxidase and C3d in plasma showed a significant inverse correlation with the hemoglobin concentration. Myeloperoxidase and C3d showed a significant positive correlation. This suggests a role for the neutrophil and the complement system in the pathophysiology of sickle cell disease.