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(Circulation. 2000;101:2446.)
© 2000 American Heart Association, Inc.

Images in Cardiovascular Medicine

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

Demonstration by the Sagittal Images of Intravascular Ultrasonography

Ryutaro Komuro, MD, PhD; Shizuya Yamashita, MD, PhD; Satoru Sumitsuji, MD; Ken-ichi Hirano, MD, PhD; Takao Maruyama, MD, PhD; Makoto Nishida, MD; Fumihiko Matsuura, MD; Akifumi Matsuyama, MD; Taizo Sugimoto, MD; Noriyuki Ouchi, MD; Naohiko Sakai, MD, PhD; Tadashi Nakamura, MD, PhD; Toru Funahashi, MD, PhD; Yuji Matsuzawa, MD, PhD

From the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, Osaka University, Osaka, Japan.

Correspondence to Ken-ichi Hirano, MD, PhD, Department of Internal Medicine and Molecular Science, Graduate School of Medicine, B5, Osaka University, 2–2, Yamadaoka, Suita, Osaka 565-0871, Japan. E-mail khirano@kb3.so-net.ne.jp

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, the diagnosis of homozygous Tangier disease was also established by biopsy of the patient’s bone marrow, showing the presence of foam cells (Figure 1).

View larger version (132K): [in this window] [in a new window]   Figure 1. Puncture sample of bone marrow stained with Sudan III and analyzed by light microscopy shows foam cells with accumulated lipid droplets (arrow). Bar=10 µm.

Our patient began having exertional chest pain in January 1997. Myocardial perfusion images with ²⁰¹Tl revealed a defect in the inferoposterior wall with an incomplete redistribution; thus, a coronary angiogram was performed in July 1997. It revealed massive and longitudinal diffuse calcifications in the 3 coronary arteries . . . [Full Text of this Article]

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