ICD-10 Code E78.3 – Hyperchylomicronemia (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Hyperchylomicronemia is E78.3.
2026 ICD-10-CM Diagnosis Code E78.3 – Hyperchylomicronemia

What it is

E78.3 identifies hyperchylomicronemia, a disorder marked by excess chylomicrons in the blood. It usually reflects severe disturbance of triglyceride metabolism and may be inherited or acquired. Check documentation for the exact cause.

Clinical signs

Clinical features vary; refer to documentation. Commonly documented findings include markedly lipemic serum, recurrent abdominal pain, pancreatitis, eruptive xanthomas, or hepatosplenomegaly. You should code only when the record clearly identifies hyperchylomicronemia.

When to use this code

Use this code when the provider diagnoses hyperchylomicronemia by name, or documents a chylomicronemia syndrome consistent with this disorder. It may appear in lipid disorder workups, genetic dyslipidemia evaluations, or follow-up notes for persistent chylomicron excess. Do not infer it from nonspecific hyperlipidemia alone.

Do not use for

Do not use E78.3 for routine hypertriglyceridemia, mixed dyslipidemia, or unspecified lipid abnormalities unless hyperchylomicronemia is specifically documented. Check documentation when the chart only mentions elevated lipids.

Coding tip

Confirm the provider uses the term hyperchylomicronemia or a clearly equivalent diagnosis before assigning E78.3.

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