What is type III hyperlipidemia?

Updated: Jul 23, 2021
  • Author: Mary Ellen T Sweeney, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Type III is known as dysbetalipoproteinemia, remnant removal disease, or broad-beta disease due to an individual’s decreased ability to convert VLDL and intermediate-density lipoprotein (IDL), a VLDL remnant, to LDL particles in the blood and because of a decreased clearance of chylomicron remnants. [4] Typically, patients with this rare condition have elevated total cholesterol (range, 300 600 mg/dL) and triglyceride levels (usually >400 mg/dL; may exceed 1000 mg/dL), [3] and these individuals are easily confused with patients with type IIb hyperlipidemia. Patients with type III hyperlipidemia have elevations in IDL.

Dysbetalipoproteinemia is the result of 2 "hits." [4] First, most affected individuals are homozygous for the apo E isoform, apo E2. Second, affected individuals usually have a metabolic disorder such as diabetes, obesity, or hypothyroidism. Consequently, those who are homozygous for apo E2 have a 1-2% risk of developing dysbetalipoproteinemia, and they are at high risk for atherosclerotic cardiovascular and peripheral vascular disease. [4] The condition responds well to treatment of the causative medical condition and to lipid-lowering medications.

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