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(Circulation. 2009;119:2741-2742.)
© 2009 American Heart Association, Inc.

Images in Cardiovascular Medicine

Tangier Disease in Severely Progressive Coronary and Peripheral Artery Disease

Tiziana Sampietro, MD; Mariarita Puntoni, PhD; Federico Bigazzi, PhD; Benedetto Pennato, PhD; Francesco Sbrana, MD; Beatrice Dal Pino, MD; Antonio Azzarà, MD; Enrico Magagnini, MD; Paolo Perossini, MD; Giovanni Cei, MD; Giovanni Bacci, MD

From the Gabriele Monasterio, CNR, Regione Toscana Foundation, Pisa (T.S., F.B., F.S.); CNR Institute of Clinical Physiology, Pisa (T.S., M.P., F.B., B.P., B.D.P.); Scuola Superiore S. Anna, Pisa (M.P., B.P.); Division of Hematology, Department of Oncology, Transplants and Advanced Technology in Medicine (A.A.), Ophthalmology Department (P.P.), and Neuroscience Department (G.C.), University of Pisa, Pisa; Cardiac Catheterization Laboratory, ASL 6, Livorno Hospital, Livorno (E.M.); and ASL 2, Campi Bisenzio, Firenze (G.B.), Italy.

Reprint requests to T. Sampietro, MD, CNR Institute of Clinical Physiology, Via Moruzzi, 1–56124, Pisa, Italy. E-mail tizisamp@ifc.cnr.it

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A 37-year-old man was first referred to our lipid clinic in December 2007 for plasma lipid alteration. He presented with large, orange tonsils (Figure 1) and hepatosplenomegaly. There was no evidence of corneal opacities nor of other ocular abnormalities, and there were no nervous system abnormalities as assessed by sensitive and motor electromyography.

View larger version (106K):   Figure 1. Orange large tonsils.

Hematologic abnormalities included thrombocytopenia and erythrocytes with altered morphology (stomatocytes; Figure 2) and function (decreased osmotic resistance). Serum levels of total cholesterol, triglycerides, and high-density lipoprotein (HDL) cholesterol were 58, 184, and 4 mg/dL, respectively; plasma apolipoprotein A-I concentrations were very low (3.9 mg/dL); and apolipoprotein A-II plasma levels were 1.7 mg/dL.

View larger version (117K):   Figure 2. Peripheral blood erythrocytes with numerous stomatocytes.

The patient’s father (58 years of age) had serum levels of total cholesterol, HDL cholesterol, and apolipoprotein A-I of 127, 25, and 99.5 mg/dL, respectively. Moreover, he had a history of premature severe coronary disease since 37 years of age that had been treated with repeated surgical revascularization.

Our patient began having exertional chest pain in March 2006 at 35 years of age. His ECG stress test was positive for myocardial ischemia, so a coronary angiogram was performed. The test revealed severe atherosclerosis in 2 vessels, 95% stenosis in the mid portion of the right coronary artery with 75% stenosis at the crux and 95% stenosis in the proximal portion of the left anterior descending coronary artery, continuing on to 2 other 50% stenoses (Figure 3).

View larger version (158K):   Figure . . . [Full Text of this Article]