The chylomicronemia syndrome is a disorder characterized by severe fasting
hypertriglyceridemia and massive accumulations of chylomicrons in plasma that can lead to
the development of eruptive xanthomas, lipemia retinalis, and pancreatitis. Genetic causes
of the syndrome include familial deficiency of lipoprotein lipase (LPL), familial deficiency of
apolipoprotein (apo) C-II, and familial inhibitor of LPL. In addition, in patients with familial
forms of moderate hypertriglyceridemia, chylomicronemia may develop when other acquired …