Hyperchylomicronemia

This code identifies a rare genetic disorder characterized by extremely high levels of chylomicrons in the blood, leading to severe hypertriglyceridemia. It results from a deficiency in lipoprotein lipase (LPL) or its activator, apolipoprotein C-II (apoC-II), impairing the body's ability to clear dietary fats.

Use this code for patients diagnosed with familial chylomicronemia syndrome (FCS) or other primary causes of severe chylomicronemia. Documentation should clearly state elevated chylomicron levels, often with triglyceride levels exceeding 1000 mg/dL, and rule out secondary causes of hypertriglyceridemia.

• Distinguish from other forms of hyperlipidemia; this code is specific to chylomicronemia.
• Ensure documentation specifies the primary nature of the hyperchylomicronemia, as opposed to secondary causes.
• Consider additional codes for associated manifestations like pancreatitis (K85.-) if present.