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A 48-year-old man was first referred to our clinic in January 1989 because of marked hypocholesterolemia with very low HDL cholesterol, anemia, and hyperbilirubinemia. He had large tonsils, corneal opacities, hepatosplenomegaly, and thrombocytopenia. Serum levels of total cholesterol, triglycerides, and HDL cholesterol were 0.72, 2.6, and 0.16 mmol/L (28, 232, and 6 mg/dL), respectively. Concentrations of apolipoproteins (apo) A-I and A-II were 1.3 and 0.9 μmol/L (3.9 and 1.5 mg/dL), respectively. His daughter’s serum levels of total cholesterol, HDL cholesterol, and apo A-I were 3.3 mmol/L, 0.64 mmol/L, and 34.7 μmol/L (128, 24, and 104 mg/dL), respectively. He was diagnosed with Tangier disease by clinical manifestations, analysis of lipoproteins, and 2D electrophoresis, which confirmed the increase of preproapo A-I. Interestingly, xanthoma in the Achilles’ tendons, which had rarely been reported in patients with Tangier disease, was observed in the patient, and the thickness was 9 mm (mean of bilateral determinations; control, 6±2 mm). Subsequently, …

Tangier Disease With Continuous Massive and Longitudinal Diffuse Calcification in the Coronary Arteries