Plasma and erythrocyte lipids in hereditary spherocytosis

Determinations were made of the concentrations of cholesterol, triglycerides and phospholipids in plasma from 18 patients with hereditary or congenital spherocytosis (HS). The plasma cholesterol and phospholipid levels of these patients were significantly depressed, whereas the triglyceride levels were normal. Phospholipids from plasma and red cell ghosts were analysed by thin-layer chromatography. The relative amount of phosphatidylethanolamine was decreased in both plasma and red cell membranes from HS patients, whereas significant alterations were not observed in the relative amounts of the other major phospholipid classes. These data show that in HS the abnormal lipid metabolism is not confined only to the red cell; it also affects the plasma lipids and therefore probably other cells as well.