The role of membrane lipids in the survival of red cells in hereditary spherocytosis

Abstract

A B S T R A C T Red cells inObstructive jaundice causes an increase in membrane lipid, primarily cholesterol, and a decrease in the osmotic fragility of normal red cells. When HS red cells are transfused into patients with obstructive jaundice they also become less osmotically fragile. Moreover, when incubated in obstructive jaundice serum, they gain cholesterol. This acquisition of membrane lipid in vitro does not result in a change in their rate of glucose utilization or sodium efflux. However, the transformation to a less spheroidal shape in vivo permits them to traverse better the splenic circulation and survive longer.

Red cell survival studies in hereditary spherocytosis

“…The homologous survival of hereditary spherocytes from one patient (R. M.) was measured both before and after his splenectomy to give an indication of the effect of red cell age on cell survival in normal recipients. The survival ti of these cells was 6.0 and 6.5 days in separate recipients (mean 6 Fig. 2 where an autologous survival ti of 12.5 days compares with the mean homologous survival of 1.5 days.…”

“…In contrast a rapid destruction of hereditary spherocytes in the normal circulation has been observed in other studies in which 'Cr survivals of ti of 6 days and 1.9 days have been reported (4,6). These results suggest Received for publication 4 September 1969 and in revised form 20 November 1969. that the low viability of hereditary spherocytes is even further reduced when these cells are transfused to normal recipients.…”

Hereditary Spherocytosis: Evaluation of 68 Children

“…These proteins are employed in the attachment of the cytoskeleton to the membrane integral domain, so any deficiency or dysfunction in them results in a loss of surface area and leads to spheroidal, osmotically fragile erythrocytes [8,9]. It is impossible for these spherocytic, fragile erythrocytes to pass through the splenic sinusoids easily.…”

Clinical and hematologic features of 300 patients affected by hereditary spherocytosis grouped according to the type of the membrane protein defect

“…[10][11][12] The deficiency or dysfunction of any of these proteins, which are involved in the attachment of the cytoskeleton to the membrane integral domain, results in a loss of surface area and leads to spheroidal, osmotically fragile cells that are selectively trapped in the spleen. 13,14 The defective protein can be detected by sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE), which allows the identification of different subsets of patients; 3,15,16 although some HS subjects remain unclassified by this technique; 16,[17][18][19] efforts to identify the protein defect by genetic analysis in unclassified cases have been unsuccessful. 20 It is usually thought that the clinical phenotype of spectrin deficiency is more severe than that of band 3 deficiency, and that the level of residual spectrin inversely correlates with the severity of anemia.…”