Hypertriglyceridemia (HTG) is a feature of numerous metabolic disorders including dyslipidemias, metabolic syndrome, and diabetes mellitus type 2 and can increase the risk of premature coronary artery disease. HTG may also be due to genetic factors (called primary HTG) and particularly the severe/extreme HTG (SEHTG), which is a usually rare genetic disorder. Even rarer are secondary cases of SEHTG caused by autoimmune disease. This review considers the causes of SEHTG, and their management including treatment with low density lipoprotein apheresis and analyzes the original findings. 1. Introduction A positive correlation between high triglycerides (TGs) concentration and coronary heart disease (CHD) has been established in numerous studies [1–11]. Hypertriglyceridemia (HTG) is prevalent in 18.6% of men and 4.2% of women between 16 and 65 years of age. The Adult Treatment Panel (ATP) III guidelines, published 13 years ago [12], described normal TGs concentration <150？mg/dL (<1.6？mmol/L), borderline-high TGs as 150 to 199？mg/dL (1.6–2.2？mmol/L), high TGs as 200 to 499？mg/dL (2.2–5.6？mmol/L), and very high TGs as >500？mg/dL (>5.6？mmol/L). However, severe/extreme hypertriglyceridemia (SEHTG) should be considered when values are greater than 1,000？mg/dL (11.2？mmol/L) because this places individuals at significant increased risk of pancreatitis. With TG values less than 1,000？mg/dL (5.6？mmol/L) one should be focused on the risk of premature CHD [13]. HTG is a feature of numerous metabolic disorders including dyslipidemias, metabolic syndrome, and diabetes mellitus type 2 (DMT2) and can increase the risk of premature CHD [14, 15]. These metabolic disorders may be caused by interactions between genetic and nongenetic factors since those subjects present usually similar clinical features (android type of obesity, ectopic fat deposition, thin arms and legs, increased waist circumference, upper body obesity, and in case of SEHTG eruptive xanthomas) [16, 17]. Visceral fat is considered to behave as ectopic fat deposition. It accumulates TGs in cases when body fat storage exceeds the capacity of fat stores. Furthermore, subjects with HTG usually present insulin resistance, hepatic steatosis, and DMT2. Thus, all the above can be called “hypertriglyceridemic phenotype.” Additionally, several studies (including ours) showed that postprandial HTG is manifested in subjects with hypertriglyceridemic phenotype [18]. HTG may also be due to genetic factors (called primary HTG) and particularly the SEHTG, which is a usually rare genetic disorder. Even rarer are secondary
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