Tangier Disease with Normal Serum Cholesterol*

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Abstract
Three patients with Tangier disease (hypo-.alpha.-lipoproteinemia) from the same family are described. One shows the clasically described lipid abnormalities of the disease, with a low serum cholesterol level, and almost absent high density lipoprotein (HDL). Two of his siblings, although demonstrable as Tangier homozygotes, have serum cholesterol levels within the normal range. This anomaly has arisen because they are also heterozygous for familial combined hyperlipidemia. The pattern of inheritance of the 2 disorders, Tangier disease and combined hyperlipidemia, appears to be unrelated.