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. The disease is Tangier disease (MONDO_0008783). Also known as: ATP-binding cassette transporter A1 deficiency, Analphalipoproteinemia, defective adenosine triphosphate-binding cassette transporter A1, familial alpha-lipoprotein deficiency, familial high density lipoprotein deficiency.