2027 ICD-10-CM Diagnosis Code E78.6Lipoprotein deficiency
ICD-10-CM Codes›E00–E89›E70-E88›E78
- Billable — Valid for Submission
- Chronic Condition
E78.6 is a billable ICD-10-CM diagnosis code for lipoprotein deficiency. 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 Other specified and unspecified nutritional and metabolic disorders.
E78.6 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
E78.6 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.
- Abetalipoproteinemia
- Acanthocytosis
- Apo A-I Giessen variant
- Apo A-I Marburg variant
- Apo A-I Milano variant
- Apo A-I variant fisheye-like syndrome
- ApoA-I Munster variant 1
- ApoA-I Munster variant 2
- ApoA-I Munster variant 3
- Apolipoprotein A-I variant disorder
- Ataxia co-occurrent and due to abetalipoproteinemia
- Autonomic neuropathy due to Tangier disease
- Chorea acanthocytosis syndrome
- Familial hypoalphalipoproteinemia
- Familial hypobetalipoproteinemia
- Familial hypobetalipoproteinemia - heterozygous form
- Familial hypobetalipoproteinemia - homozygous form
- Familial hypolipoproteinemia
- Familial lecithin cholesterol acyltransferase deficiency
- Familial lipoprotein deficiency
- Fish-eye disease
- Hereditary acanthocytosis
- High density lipoprotein below reference range
- High density lipoprotein deficiency
- Hypoalphalipoproteinemia
- Hypo-beta-lipoproteinemia
- Hypocholesterolemia
- Hypolipoproteinemia
- Inborn error of lipoprotein metabolism
- Lecithin cholesterol acyltransferase deficiency
- Lipoprotein below reference range
- Lipoprotein deficiency disorder
- Low density lipoprotein cholesterol below reference range
- McLeod neuroacanthocytosis syndrome
- Ophthalmoplegia due to abetalipoproteinemia
- Progressive chorea
- Tangier disease
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E78.6: 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.
- Abetalipoproteinemia
- Depressed HDL cholesterol
- High-density lipoprotein deficiency
- Hypoalphalipoproteinemia
- Hypobetalipoproteinemia (familial)
- Lecithin cholesterol acyltransferase deficiency
- Tangier disease
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 E78.6, its category, or a range that includes it.
Excludes2 2
These codes carry an Excludes2 note naming E78.6: 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.
Abetalipoproteinemia E78.6
Acanthocytosis E78.6
Analphalipoproteinemia E78.6
HDL cholesterol E78.6
Disease, diseased See Also: Syndrome;
Tangier E78.6
Disorder (of) See Also: Disease;
Glomerulonephritis See Also: Nephritis; N05.9
Hypoalphalipoproteinemia E78.6
Tangier disease E78.6
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Abetalipoproteinemia
an autosomal recessive disorder of lipid metabolism. it is caused by mutation of the microsomal triglyceride transfer protein that catalyzes the transport of lipids (triglycerides; cholesterol esters; phospholipids) and is required in the secretion of beta-lipoproteins (low density lipoproteins or ldl). features include defective intestinal lipid absorption, very low serum cholesterol level, and near absent ldl.Hypoalphalipoproteinemias
conditions with abnormally low levels of alpha-lipoproteins (high-density lipoproteins) in the blood. hypoalphalipoproteinemia can be associated with mutations in genes encoding apolipoprotein a-i; lecithin cholesterol acyltransferase; and atp-binding cassette transporters.Hypobetalipoproteinemias
conditions with abnormally low levels of beta-lipoproteins (low density lipoproteins or ldl) in the blood. it is defined as ldl values equal to or less than the 5th percentile for the population. they include the autosomal dominant form involving mutation of the apolipoproteins b gene, and the autosomal recessive form involving mutation of the microsomal triglyceride transfer protein. all are characterized by low ldl and dietary fat malabsorption.Hypolipoproteinemias
conditions with abnormally low levels of lipoproteins in the blood. this may involve any of the lipoprotein subclasses, including alpha-lipoproteins (high-density lipoproteins); beta-lipoproteins (low-density lipoproteins); and prebeta-lipoproteins (very-low-density lipoproteins).Tangier Disease
an autosomal recessively inherited disorder caused by mutation of atp-binding cassette transporters involved in cellular cholesterol removal (reverse-cholesterol transport). it is characterized by near absence of alpha-lipoproteins (high-density lipoproteins) in blood. the massive tissue deposition of cholesterol esters results in hepatomegaly; splenomegaly; retinitis pigmentosa; large orange tonsils; and often sensory polyneuropathy. the disorder was first found among inhabitants of tangier island in the chesapeake bay, md.
Patient EducationClinical
Lipid Metabolism Disorders
Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E78.6 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E78.6Overview
What is the ICD-10 code for lipoprotein deficiency?
The ICD-10-CM code for lipoprotein deficiency is E78.6 (sometimes written as E786). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E78.6 (Disorders of lipoprotein metabolism and other lipidemias) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report lipoprotein deficiency on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E78.6 group to?
When lipoprotein deficiency 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 E78.6 be reported with a code from E34.5?
Not as a rule. The Excludes1 note at block E70-E88, which applies to E78.6, 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 E78.6?
Under the General Equivalence Mappings, lipoprotein deficiency converts to ICD-9-CM 272.5 (lipoprotein deficiencies). The mapping is a direct match.
Does E78.6 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage: E78.6 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).
