E78.1

Pure hypertriglyceridemia

Pure hypertriglyceridemia is a metabolic disorder characterized by isolated elevations of plasma triglycerides, typically without a significant elevation in cholesterol levels. This condition aligns with Fredrickson Type IV hyperlipoproteinemia, involving an increase in very-low-density lipoproteins (VLDL). It is considered 'pure' when it is not part of [Mixed hyperlipidemia](/icd10/e78-2). Clinical significance arises primarily when triglyceride levels exceed 500-1,000 mg/dL, as this dramatically increases the risk for [Acute pancreatitis](/icd10/k85-9). While often primary (genetic), it is frequently secondary to or exacerbated by factors such as [Obesity](/icd10/e66-9), uncontrolled [Type 2 diabetes mellitus](/icd10/e11-9), excessive alcohol consumption, and diets high in refined carbohydrates.

Clinical Symptoms

  • Often asymptomatic in mild to moderate cases
  • Epigastric pain or acute abdominal pain if pancreatitis occurs
  • Eruptive xanthomas (small, yellowish-orange papules on the trunk or extremities)
  • Lipemia retinalis (milky appearance of the retinal arteries and veins)
  • Hepatosplenomegaly
  • Metabolic syndrome symptoms including insulin resistance

Common Causes

  • Genetic mutations (e.g., LPL deficiency, APOA5, or APOC2 mutations)
  • Excessive dietary intake of sugars and refined carbohydrates
  • High alcohol consumption
  • Sedentary lifestyle and [Obesity](/icd10/e66-9)
  • Medications such as thiazide diuretics, non-selective beta-blockers, and estrogens
  • Poorly controlled [Type 2 diabetes mellitus](/icd10/e11-9)

Documentation & Coding Tips

Document the specific triglyceride levels to justify medical necessity for aggressive therapy or monitoring for [Acute pancreatitis](/icd10/k85-9).

Example: Patient with fasting triglycerides of 1,150 mg/dL, presenting with abdominal tenderness.

Verify if the hyperlipidemia is truly 'pure' by checking the LDL-C and Total Cholesterol; if both are elevated, use [Mixed hyperlipidemia](/icd10/e78-2).

Example: Lipid panel shows TG 600, LDL 95, HDL 30; coded as E78.1 rather than E78.2.

Identify and code any underlying causes if known, such as [Alcoholic liver disease](/icd10/k70-9) or medication-induced states.

Example: Hypertriglyceridemia due to secondary effects of chronic alcohol abuse.

Related Diagnoses

Hierarchy