The Effect of Plasma Transfusion on the Plasma Cholesterol Esters in Patients with Familial Plasma Lecithin: Cholesterol Acyltransferase Deficiency

Abstract

Two patients with familial plasma lecithin: cholesterol acyltransferase deficiency (familial plasma cholesterol ester deficiency) received about 750 ml of fresh ACD-plasma. Most of the transfused lecithin: cholesterol acyltransferase was recovered in the plasma of the patients after the transfusion. A gradual increase in the plasma cholesterol esters took place reaching a maximum 4–6 days after the transfusion. Most of the cholesterol esters formed had unsaturated acyl groups, suggesting that the fatty acids had been transferred from the 2-position of lecithin to the cholesterol. Concomitantly with the increase in cholesterol esters, the concentration of lysolecithin increased in plasma. No significant change in the red cell lipid pattern could be demonstrated.

Key Words:

• Cholesterol
• cholesterol esters
• enzymes
• erythrocytes
• fatty acids
• inborn error of lipid metabolism
• lechithin
• lecithin: cholesterol acyltransferase
• lysolecithin
• phospholipids