2026 ICD-10-CM Diagnosis Code E78.6Lipoprotein deficiency

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.6
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.6 is a billable ICD-10-CM diagnosis code for lipoprotein deficiency. 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 Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E78.6
Billable Status
Yes — Valid for Submission
Code Describes
Lipoprotein deficiency
Short Description
Lipoprotein deficiency
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 CodeE78.6Lipoprotein deficiency

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 deficiency
  • 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
  • Tangier disease

Tabular List NotesGuidance

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

Inclusion Terms

  • Abetalipoproteinemia
  • Depressed HDL cholesterol
  • High-density lipoprotein deficiency
  • Hypoalphalipoproteinemia
  • Hypobetalipoproteinemia (familial)
  • Lecithin cholesterol acyltransferase deficiency
  • Tangier disease

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.

    • Abetalipoproteinemia
    • Acanthocytosis
    • Analphalipoproteinemia
    • Bassen-Kornzweig disease or syndrome
    • Deficiency, deficient
      • lecithin cholesterol acyltransferase
    • Deficiency, deficient
      • lipoprotein (familial) (high density)
    • Depressed
      • HDL cholesterol
    • Disease, diseased
      • Tangier
    • Disorder(of)
      • glomerular (in)
        • familial lecithin cholesterol acyltransferase deficiency
    • Disorder(of)
      • lipoprotein
        • deficiency (familial)
    • Dyslipidemia
      • depressed HDL cholesterol
    • Glomerulonephritis
      • in (due to)
        • lecithin cholesterol acyltransferase deficiency
    • Hypoalphalipoproteinemia
    • Hypobetalipoproteinemia(familial)
    • Hypolipoproteinemia(alpha) (beta)
    • Tangier disease

Clinical ClassificationClinical

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

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

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.

  • Hypobetalipoproteinemia, Familial, Apolipoprotein B

    an autosomal dominant disorder of lipid metabolism. it is caused by mutations of apolipoproteins b, main components of chylomicrons and beta-lipoproteins (low density lipoproteins or ldl). features include abnormally low ldl, normal triglyceride level, and dietary fat malabsorption.

  • Lecithin Cholesterol Acyltransferase Deficiency

    an autosomal recessive disorder of lipoprotein metabolism caused by mutation of lecithin cholesterol acyltransferase gene. it is characterized by low hdl-cholesterol levels, and the triad of corneal opacities; hemolytic anemia; and proteinuria with renal failure.

  • 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.

  • Hypoalphalipoproteinemia

    a metabolic disorder characterized by deficiency of high density (alpha) lipoprotein in the blood.

  • Abetalipoproteinemia

    an autosomal recessive disorder characterized by defective absorption of dietary fat, cholesterol and fat-soluble vitamins. it results in multiple vitamin deficiencies. signs and symptoms include failure to thrive, diarrhea, steatorrhea, acanthocytosis and ataxia.

  • ABCA1 wt Allele|ABC-1|ABC1|ATP-Binding Cassette, Sub-Family A (ABC1), Member 1 wt Allele|ATP-Binding Cassette, Subfamily A, Member 1 Gene|CERP|HDLDT1|TGD|Tangier Disease Gene

    human abca1 wild-type allele is located in the vicinity of 9q31 and is approximately 147 kb in length. this allele, which encodes atp-binding cassette sub-family a member 1 protein, is involved in intracellular cholesterol transport. mutation of the gene is associated with high density lipoprotein deficiency type 2 (autosomal dominant) and tangier disease.

  • Tangier Disease

    a rare, autosomal recessive inherited disorder of cholesterol transport, resulting in severe reduction of the amount of high density lipoprotein in the plasma and accumulation of cholesterol esters in the tissues. signs and symptoms include large tonsils, hepatosplenomegaly, lymphadenopathy, and hypocholesterolemia.

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.

ICD-9-CM
272.5 Lipoprotein deficiencies
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 E78.6Overview

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, 2025 through September 30, 2026.

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.

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.