2027 ICD-10-CM Diagnosis Code E78.3Hyperchylomicronemia
ICD-10-CM Codes›E00–E89›E70-E88›E78
- Billable — Valid for Submission
- Chronic Condition
E78.3 is a billable ICD-10-CM diagnosis code for hyperchylomicronemia. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Disorders of lipid metabolism.
E78.3 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 47 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E78.3 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E78.3: its own notes plus those printed at E78, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Chylomicron retention disease
- Fredrickson's hyperlipoproteinemia, type I or V
- Hyperlipidemia, group D
- Mixed hyperglyceridemia
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E78.3, its category, or a range that includes it.
Excludes2 2
These codes carry an Excludes2 note naming E78.3: its condition is not part of those codes, and both may be reported when the patient has both.
- E88.0 Disorders of plasma-protein metabolism, not elsewhere classifieddisorder of lipoprotein metabolism (E78.-) names E78.-, which includes this code
- Block R70-R79 Abnormal findings on examination of blood, without diagnosisabnormalities of lipids (E78.-) names E78.-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Disease, diseased See Also: Syndrome;
Lipemia See Also: Hyperlipidemia;
retina, retinalis E78.3
Prebetalipoproteinemia (acquired) (essential) (familial) (hereditary) (primary) (secondary) E78.1
with chylomicronemia E78.3
Syndrome See Also: Disease;
Bürger-Grütz E78.3
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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.
Code HistoryHistory
Questions About E78.3Overview
What is the ICD-10 code for hyperchylomicronemia?
The ICD-10-CM code for hyperchylomicronemia is E78.3 (sometimes written as E783). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
What MS-DRG does E78.3 group to?
When hyperchylomicronemia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E78.3 be reported with a code from E34.5?
Not as a rule. The Excludes1 note at block E70-E88, which applies to E78.3, lists "androgen insensitivity syndrome (E34.5-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
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.
Does E78.3 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage: E78.3 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 47 (Disorders of Lipoid Metabolism).
