Observations on plasma and red cell lipids in hereditary spherocytosis.

Plasma lipids and red cell lipids were determined in hereditary spherocytosis (HS), 15 unsplenectomized and eight splenectomized patients. Plasma lipids (cholesterol: total, free and high density lipoproteins, free fatty acids, and phospholipids) were markedly decreased in HS, especially in the unsplenectomized patients. Concomitantly, red cell membrane lipids (free cholesterol and phospholipids: total, phosphatidyl ethanolamine, sphingomyelin, phosphatidyl choline, and lysophosphatidyl choline) were also diminished. The plasma lipids stay low even after splenectomy although partially restored. Thus, decreased membrane surface, a hallmark of HS, could be partly attributed to the diminished membrane lipids induced by plasma lipid abnormalities in the patients.