Tangier disease

Tangier disease (TD) is a rare lipoprotein metabolism disorder characterized biochemically by an almost complete absence of plasma high-density lipoproteins (HDL), and clinically by liver, spleen, lymph node and tonsil enlargement along with peripheral neuropathy in children and adolescents, and, occasionally, cardiovascular disease in adults.

Hepatosplenomegaly

Simultaneous enlargement of the liver and spleen.


Total: 2

                       


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PMID (PMCID)
29563393
(6193190)
 MALE Middle Aged
Accelerated Atherogenicity in Tangier Disease.
Muratsu J, Koseki M, Masuda D, Yasuga Y, Tomoyama S, Ataka K, Yagi Y, Nakagawa A, Hamada H, Fujita S, Hattori H, Ohama T, Nishida M, Hiraoka H, Matsuzawa Y, Yamashita S.
J Atheroscler Thromb. 2018;25(10):1076-1085.
Orange-colored tonsils, mild hepatosplenomegaly and very low levels of serum high-density lipoprotein cholesterol (HDL-C) were observed, and the patient was diagnosed with Tangier disease.
7082413
 MALE Middle Aged
Hypoalpha-hyperbeta-lipoproteinemia in a patient with coronary artery disease and occlusive peripheral arterial disease.
Toth L, Koenig W.
Atherosclerosis. 1982;42(1):121-4.
Unlike the clinical manifestations of Tangier disease, our patient did not show skin lesion, abnormal tonsils, hepatosplenomegaly, peripheral neurologic abnormalities or corneal deposits.