Autoimmune Hyperchylomicronemia

Abstract

AMONG the hyperchylomicronemias, familial deficiencies of lipoprotein lipase (LPL) and of apolipoprotein C-II are rare variants in which the underlying biochemical defects are relatively well defined. The diseases are characterized by a deficiency of LPL, which is the key enzyme in the hydrolysis of triglyceride-rich lipoproteins, or a defect in apolipoprotein C-II, a cofactor of LPL.1 2 3 4 Hyperchylomicronemia may also be a primary characteristic of type V familial hyperlipoproteinemia,5 or it may be secondary to a variety of conditions, such as diabetes mellitus, alcoholism, chronic renal failure, or hypothyroidism.6 7 8 9 10 11 The mechanism of secondary hyperchylomicronemia has not been fully elucidated.We describe . . .