Individual variability in response to a single sickling event for normal, sickle cell, and sickle trait erythrocytes

摘要: Hemoglobin S (Hb-S) polymerization is the primary event in sickle cell disease causing irreversible damage to red blood (RBC) membranes over repeated cycles. A single triggered by a hypoxic environment was reported result reversibly (upon reoxygenation) decreased RBC deformability and increased mechanical fragility (MF). Individualized responses have not been reported, although can vary significantly even among healthy individuals. This study evaluates individual variability response hypoxia-induced sickling event, through changes MF. Blood drawn from 10 normal (AA), 11 (SS), 7 trait (AS) subjects—with Hb-S fraction, osmotic fragility, medical history also collected. Mechanical stress applied using bead mill at 50-Hz oscillation for 0.5–30 minutes. MF profiles here give percent hemolysis upon successive durations of stressing. measured AA, SS, AS cells—each equilibrated (1) with air, (2) nitrogen an anaerobic chamber, (3) air after event. While AA subjects exhibited different hypoxia, all cases there recovery close initial values on reoxygenation. For subjects, reoxygenation observed only about half cases. Fragility SS cells hypoxia reoxygenation, variable magnitude recovery. The indicates that some are potentially higher risk of irreversible membrane damage.