Abnormality of serum lipoprotein and spleen glycosphingolipids in a case of hereditary spherocytosis.

A patient with hereditary spherocytosis gave rise to lower levels of both erythrocyte and serum cholesterols and serum lipoproteins than normal subjects. Abnormal shape of the serum lipoprotein particles was observed by electron-microscopic examination as well as the spheroidal shape of red cells. Lipids of fresh spleen tissue of the patient after splenectomy for the treatment of splenomegaly were extracted, isolated and analyzed. Particularly, different glycosphingolipids were also chemically identified and analyzed. The total lipids accounted for 10.7% of the dry tissue weight and consisted of cholesterol (32.2%), simple lipids (5.4%),phospholipids (58.3%) and glycosphingolipids (3.6%). Particularly, the glycosphingolipids were composed of monohexosyl ceramide (9.0%), lactosyl ceramide (31.2%), digalactosylglucosyl ceramide (9.2%), globoside I (27.6%) and hematoside (23.0%). It was found that the globoside I and hematoside increased remarkably in the spleen of patient with hereditary spherocytosis and that monohexosyl ceramide contained not only glucosyl ceramide, but also galactosyl ceramide at the ratio of 4:1.