lactoferrin and Anemia--Sickle-Cell

Topics: Agammaglobulinemia; Anemia, Sickle Cell; Chemotaxis; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Immunity, Cellular; Immunoglobulin E; Immunologic Deficiency Syndromes; Infections; Job Syndrome; Lactoferrin; Leukocytes; Male; Muramidase; Opsonin Proteins; Peroxidase; Phagocytes; Phagocytosis; Staphylococcus

Topics: Anemia, Sickle Cell; Hemoglobin A; Hemoglobin, Sickle; Humans; Infections; Lactoferrin; Reference Values

Vascular occlusion is the main cause of the morbidity and mortality observed in patients with sickle cell disease (SCD). Increasing evidence indicates that (activated) neutrophils could play an important role in the initiation and propagation of vaso-occlusive processes in SCD. In this study, the activation state of neutrophils in sickle cell patients was analyzed by determining the level of expression of neutrophil antigens such as CD62L, CD11b, CD66b, CD63, and Fcgamma receptors. We also analyzed plasma levels of lactoferrin, elastase, soluble (s)CD16 (sFcgammaRIII), and serum levels of soluble (s)CD62L (sL-selectin) as neutrophil activation markers in these patients. Significant differences were observed in the activation state of neutrophils in non-symptomatic sickle cell patients compared to healthy HbAA controls as exemplified by significant decrease in L-selectin expression, enhanced expression of CD64, and increased levels of soluble markers like sL-selectin, elastase, and sCD16. During vaso-occlusive crisis the differences were even more pronounced. These results show neutrophils to be activated in sickle cell patients, suggesting a role of importance in the pathophysiology of sickle cell disease.

Topics: Abdomen, Acute; Acute Disease; Anemia, Sickle Cell; Antigens, CD; Antigens, Surface; Biomarkers; Cytokines; Humans; Immunophenotyping; Lactoferrin; Leukocyte Elastase; Neutrophils; Receptors, IgG; Respiratory Burst; Vascular Diseases

Parameters of host defence were investigated in 30 patients with sickle cell disease (SCD). A newly devised perfusion system was used to study the kinetics in whole blood of leucocyte adherence, phagocytosis, killing and solubilization of a mixture of Staph. aureus and Str. pneumoniae, and secretion of lactoferrin. A skin window technique was used to examine the accumulation of leucocytes at inflammatory foci and their subsequent rate of movement through a filter. Serum concentrations of C3, C4, total haemolytic complement and immunoglobulins were also measured. The rate of neutrophil migration into filters was slightly reduced in patients with SCD. The proportion of monocytes that emigrated from the skin windows and their rate of migration were markedly diminished. The adhesion of neutrophils and their ability to kill staphylococci were also reduced, particularly in patients of the haemoglobin (Hb) SS and Hb S-beta-thalassaemia genotypes. Neutrophil function was mostly impaired in patients with the greatest frequency of bacterial infection. The rate of clearance of pneumococci was related to the concentration of type specific immunoglobulin G but not M. Serum concentrations of immunoglobulins and complement were normal. We were unable to define a defect of host defence of sufficient magnitude to explain the susceptibility of these patients to severe infection.

Topics: Anemia, Sickle Cell; Bacteriolysis; Blood Bactericidal Activity; Cell Movement; Complement System Proteins; Humans; Immunity; Immunoglobulins; Lactoferrin; Phagocytes; Phagocytosis; Skin Window Technique; Staphylococcus aureus

Topics: Anemia, Sickle Cell; Child; Child, Preschool; Humans; Lactoferrin; Lactoglobulins; Leukocytosis; Neutrophils; Prostaglandins E