2026 ICD-10-CM Diagnosis Code E78.3Hyperchylomicronemia

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.3
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.3 is a billable ICD-10-CM diagnosis code for hyperchylomicronemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Disorders of lipid metabolism.

Code Identity

ICD-10-CM Code
E78.3
Billable Status
Yes — Valid for Submission
Code Describes
Hyperchylomicronemia
Short Description
Hyperchylomicronemia
Same as the full description in the CMS dataset.
Parent Code
E78
Disorders of lipoprotein metabolism and other lipidemias

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE78Disorders of lipoprotein metabolism and other lipidemias
This CodeE78.3Hyperchylomicronemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Chylomicron retention disease
  • Chylomicronemia syndrome
  • Endogenous hyperlipidemia
  • Familial apolipoprotein C-II deficiency
  • Familial chylomicronemia syndrome
  • Familial hyperchylomicronemia
  • Familial hypertriglyceridemia
  • Familial hypobetalipoproteinemia
  • Familial lipoprotein deficiency
  • Familial lipoprotein lipase deficiency with type I phenotype
  • Familial lipoprotein lipase deficiency with type V phenotype
  • Familial type 5 hyperlipoproteinemia
  • Hyperlipidemia with lipid deposition in skin
  • Hyperlipoproteinemia
  • Hyperlipoproteinemia, type I
  • Inborn error of lipoprotein metabolism
  • Intestinal malabsorption
  • Intestinal malabsorption of fat
  • Lipidemia retinalis
  • Primary acquired chylomicronemia
  • Primary chylomicronemia
  • Primary genetic hyperlipidemia
  • Primary hypertriglyceridemia
  • Xanthoma due to abnormality of lipid metabolism
  • Xanthoma due to primary chylomicronemia

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Chylomicron retention disease
  • Fredrickson's hyperlipoproteinemia, type I or V
  • Hyperlipidemia, group D
  • Mixed hyperglyceridemia

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Bürger-Grütz disease or syndrome
    • Chylomicronemia(fasting) (with hyperprebetalipoproteinemia)
    • Disease, diseased
      • Bürger-Grütz (essential familial hyperlipemia)
    • Disease, diseased
      • chylomicron retention
    • Frederickson's hyperlipoproteinemia, type
      • I and V
    • Hepatosplenomegaly
      • hyperlipemic (Bürger-Grütz type)
    • Hyperchylomicronemia(familial) (primary)
    • Hyperchylomicronemia(familial) (primary)
      • with hyperbetalipoproteinemia
    • Hyperglyceridemia(endogenous) (essential) (familial) (hereditary) (pure)
      • mixed
    • Hyperlipemia, hyperlipidemia
      • group
        • D
    • Hyperlipoproteinemia
      • Fredrickson's type
        • I
    • Hyperlipoproteinemia
      • Fredrickson's type
        • V
    • Lipemia
      • retina, retinalis
    • Lipidosis
      • hepatosplenomegalic
    • Prebetalipoproteinemia(acquired) (essential) (familial) (hereditary) (primary) (secondary)
      • with chylomicronemia
    • Syndrome
      • Bürger-Grütz
    • Xanthoma(s), xanthomatosis (primary) (familial) (hereditary)
      • with
        • hyperlipoproteinemia
          • Type I
    • Xanthoma(s), xanthomatosis (primary) (familial) (hereditary)
      • with
        • hyperlipoproteinemia
          • Type V

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END010
Disorders of lipid metabolism
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Hyperlipoproteinemia Type I

    an inherited condition due to a deficiency of either lipoprotein lipase or apolipoprotein c-ii (a lipase-activating protein). the lack of lipase activities results in inability to remove chylomicrons and triglycerides from the blood which has a creamy top layer after standing.

  • Hyperlipoproteinemia Type II

    a group of familial disorders characterized by elevated circulating cholesterol contained in either low-density lipoproteins alone or also in very-low-density lipoproteins (pre-beta lipoproteins).

  • Hyperlipoproteinemia Type III

    an autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (idl or broad-beta-lipoprotein). idl has a cholesterol to triglycerides ratio greater than that of very-low-density lipoproteins. this disorder is due to mutation of apolipoproteins e, a receptor-binding component of vldl and chylomicrons, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.

  • Hyperlipoproteinemia Type IV

    a hypertriglyceridemia disorder, often with autosomal dominant inheritance. it is characterized by the persistent elevations of plasma triglycerides, endogenously synthesized and contained predominantly in very-low-density lipoproteins (pre-beta lipoproteins). in contrast, the plasma cholesterol and phospholipids usually remain within normal limits.

  • Hyperlipoproteinemia

    an elevated concentration of lipoproteins.

  • Hyperlipoproteinemia, Type I|Familial Essential Hyperlipemia|Familial Hyperchylomicronemia|Hyperlipoproteinemia Type 1A|Lipoprotein Lipase Deficiency

    a genetic disorder of lipoprotein metabolism caused by mutations in the lpl and apolipoprotein (apo) c-ii genes. it is characterized by increased levels of chylomicrons and triglycerides in the blood.

  • Hyperlipoproteinemia, Type I|Familial Essential Hyperlipemia|Familial Hyperchylomicronemia|Hyperlipoproteinemia Type 1A|Lipoprotein Lipase Deficiency

    a genetic condition caused by mutation(s) in the lpl gene, encoding lipoprotein lipase, resulting in reduced activity of the enzyme lipoprotein lipase.

  • Hyperlipoproteinemia, Type II|Type II Hyperlipidemia

    an inheritable form of hyperlipidemia, in which there are excess lipids in the blood.

  • Hyperlipoproteinemia, Type IIa|FH|Familial Hypercholesterolemia|Hyperlipidemia Type IIa|Type IIa Hyperlipidemia

    an autosomal dominant inherited disorder characterized by very high levels of low-density lipoprotein cholesterol (ldl-c) and total cholesterol in the blood. it is usually caused by mutations in the ldlr gene which is located on the short arm of chromosome 19.

  • Hyperlipoproteinemia, Type IIb|Familial Combined Hyperlipidemia|Hyperlipidemia Type IIb|Type IIb Hyperlipidemia|Type IIb Hyperlipoproteinemia

    a disorder of lipoprotein metabolism characterized by high levels of cholesterol and triglycerides in the blood. it is caused by elevation of low density and very low density lipoproteins.

Patient EducationClinical

Cholesterol

Cholesterol is a waxy, fat-like substance that's found in all the cells in your body. Your body needs some cholesterol to make hormones, vitamin D, and substances that help you digest foods. Your body makes all the cholesterol it needs. Cholesterol is also found in foods from animal sources, such as egg yolks, meat, and cheese.

The full article covers:

  • What is cholesterol?
  • What are HDL, LDL, and VLDL?
  • What causes high cholesterol?
  • What can raise my risk of high cholesterol?
  • What other health problems can high cholesterol cause?
  • How is high cholesterol diagnosed?
  • How can I lower my cholesterol?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E78.3 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
272.3 Hyperchylomicronemia
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E78.3Overview

Is E78.3 (Disorders of lipoprotein metabolism and other lipidemias) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hyperchylomicronemia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E78.3?

Under the General Equivalence Mappings, hyperchylomicronemia converts to ICD-9-CM 272.3 (hyperchylomicronemia). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.