ICD-10-CM 2027
ICD-10 code for Hyperchylomicronemia
From the official ICD-10-CM alphabetic index entry “Hyperchylomicronemia (familial) (primary)”. Page updated September 29, 2026.
About coding Hyperchylomicronemia
The ICD-10-CM code for Hyperchylomicronemia is E78.3 (Hyperchylomicronemia). The official index lists 1 more specific code, so check the documentation for details such as type, cause, site or severity before settling on E78.3.
Within E78 (disorders of lipoprotein metabolism and other lipidemias), E78.3 is specifically for Hyperchylomicronemia. Related codes cover pure hypercholesterolemia (E78.0), pure hyperglyceridemia (E78.1), mixed hyperlipidemia (E78.2), other hyperlipidemia (E78.4), hyperlipidemia, unspecified (E78.5), lipoprotein deficiency (E78.6), disorders of bile acid and cholesterol metabolism (E78.7), other disorders of lipoprotein metabolism (E78.8) and 1 more.
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific Hyperchylomicronemia codes (official index)
- with hyperbetalipoproteinemia E78.3 — Hyperchylomicronemia
Common questions about Hyperchylomicronemia ICD-10 codes
What is the ICD-10 code for Hyperchylomicronemia?
- E78.3 — Hyperchylomicronemia. More specific codes apply when the documentation supports them: E78.3.
Is E78.3 billable?
- Yes. E78.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.3 as a principal diagnosis.
Is E78.3 a CC or MCC?
- No. E78.3 is neither a CC nor an MCC under MS-DRG v44.0.