Deficiency of apolipoproteins A-I and C-III and severe coronary heart disease.

A 55-year-old woman with severe coronary arteriosclerosis and skin xanthomas is described. The patient had a normal total cholesterol level of 168 mg/dl. Her level of high-density lipoprotein cholesterol was markedly reduced (3 mg/dl). On apolipoprotein analysis, apolipoproteins A-I and C-III were not detectable, and the level of apolipoprotein A-II was found to be at a low level (3.5 mg/dl). This case may possibly belong to a distinct new disease entity of deficiencies of apolipoprotein A-I and C-III in which coronary arteriosclerosis appears prematurely and severely.