[Mechanisms of enhanced erythrocyte destruction in patients with erythrocyte membrane disorders].

Factors causing erythrocyte destruction in patients with congenital erythrocyte membrane disorders include 1) decreased cell deformability, 2) increased cell viscosity, 3) decreased membrane stability, 4) increased intracellular Ca2+ concentrations, 5) changes in membrane permeability, 6) extracellular conditions such as hypoxia and acidic pH, or 7) hypersplenism. erythrocytes with shape changes due to abnormality of membrane skeleton lose deformability to negotiate the interendothelial slits into the splenic venous sinus, and stagnate within the cordal space where metabolic stress reduces membrane stability. Thereafter, erythrocytes are ingested by cordal macrophages, resulting in extravascular hemolysis.