2022 ICD-10-CM Diagnosis Code E78.6

Lipoprotein deficiency

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
  • E78.6 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2022 edition of ICD-10-CM E78.6 became effective on October 1, 2021.
  • This is the American ICD-10-CM version of E78.6 - other international versions of ICD-10 E78.6 may differ.
Applicable To
  • Abetalipoproteinemia
  • Depressed HDL cholesterol
  • High-density lipoprotein deficiency
  • Hypoalphalipoproteinemia
  • Hypobetalipoproteinemia (familial)
  • Lecithin cholesterol acyltransferase deficiency
  • Tangier disease
The following code(s) above E78.6 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E78.6:
  • E00-E89
    2022 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • 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.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2022 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Type 2 Excludes
    • Ehlers-Danlos syndromes (Q79.6-)
    Metabolic disorders
  • E78
    ICD-10-CM Diagnosis Code E78

    Disorders of lipoprotein metabolism and other lipidemias

      2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of lipoprotein metabolism and other lipidemias
Approximate Synonyms
  • Abetalipoproteinemia
  • Familial hypoalphalipoproteinemia
  • Familial hypobetalipoproteinemia
  • Familial lipoprotein lipase deficiency
  • High density lipoid deficiency
  • High density lipoprotein (hdl) deficiency
  • High density lipoprotein deficiency
  • Hypo-beta-lipoproteinemia
  • Hypoalphalipoproteinemia, familial
  • Hypobetalipoproteinemia
  • Hypobetalipoproteinemia, familial
  • Lipoprotein deficiency disorder
Clinical Information
  • A disorder of lipoprotein metabolism caused by mutations in the lcat gene. It is characterized by deficiency of the enzyme lecithin cholesterol acyltransferase. It is manifested with corneal opacity, hemolytic anemia, and proteinuria.
  • A metabolic disorder characterized by deficiency of high density (alpha) lipoprotein in the blood.
  • 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.
  • 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.
  • 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.
  • 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.
  • An autosomal recessively inherited disorder caused by mutation of lecithin cholesterol acyltransferase that facilitates the esterification of lipoprotein cholesterol and subsequent removal from peripheral tissues to the liver. This defect results in low hdl-cholesterol level in blood and accumulation of free cholesterol in tissue leading to a triad of corneal opacity, hemolytic anemia (anemia, hemolytic), and proteinuria.
  • Autosomal recessive disorder of lipoprotein and lipid metabolism characterized by absence in plasma of normal high-density lipoprotein, deficiency of apolipoproteins ai and aii, low to normal low-density lipoproteins, high triglycerides and accumulation in body tissues of cholesteryl esters.
  • 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.
  • 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).
  • Disease characterized by abnormally low levels of plasma lecithin cholesterol acyl transferase; clinical manifestations include corneal opacity, anemia, and proteinuria.
  • Disorder of lipid metabolism inherited as an autosomal recessive trait characterized by the near absence of apolipoprotein b and apob containing lipoproteins in plasma; microsomal triglyceride transfer protein is deficient or absent in enterocytes; clinical and laboratory findings include acanthocytosis, hypocholesterolemia, peripheral neuropathy, posterior column degeneration, ataxia, and steatorrhea; intellectual abilities may also be impaired.
  • Presence of abnormally low levels of lipoproteins in the serum.
ICD-10-CM E78.6 is grouped within Diagnostic Related Group(s) (MS-DRG v39.0):
  • 642 Inborn and other disorders of metabolism

Convert E78.6 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change

Diagnosis Index entries containing back-references to E78.6:
  • Abetalipoproteinemia E78.6
  • Acanthocytosis E78.6
  • Analphalipoproteinemia E78.6
  • Bassen-Kornzweig disease or syndrome E78.6
  • Deficiency, deficient
    • lecithin cholesterol acyltransferase E78.6
    • lipoprotein E78.6 (familial) (high density)
  • Depressed
    • HDL cholesterol E78.6
  • Disease, diseased - see also Syndrome
    • Tangier E78.6
  • Disorder (of) - see also Disease
    • glomerular (in) N05.9
      ICD-10-CM Diagnosis Code N05.9

      Unspecified nephritic syndrome with unspecified morphologic changes

        2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
      • familial lecithin cholesterol acyltransferase deficiency E78.6
    • lipoprotein
      • deficiency E78.6 (familial)
  • Dyslipidemia E78.5
    ICD-10-CM Diagnosis Code E78.5

    Hyperlipidemia, unspecified

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    • depressed HDL cholesterol E78.6
  • Glomerulonephritis N05.9
    - see also Nephritis
    ICD-10-CM Diagnosis Code N05.9

    Unspecified nephritic syndrome with unspecified morphologic changes

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    • in (due to)
      • lecithin cholesterol acyltransferase deficiency E78.6
  • Hypoalphalipoproteinemia E78.6
  • Hypobetalipoproteinemia E78.6 (familial)
  • Hypolipoproteinemia E78.6 (alpha) (beta)
  • Tangier disease E78.6

ICD-10-CM Codes Adjacent To E78.6
E78.0 Pure hypercholesterolemia
E78.00 …… unspecified
E78.01 Familial hypercholesterolemia
E78.1 Pure hyperglyceridemia
E78.2 Mixed hyperlipidemia
E78.3 Hyperchylomicronemia
E78.4 Other hyperlipidemia
E78.41 Elevated Lipoprotein(a)
E78.49 Other hyperlipidemia
E78.5 Hyperlipidemia, unspecified
E78.6 Lipoprotein deficiency
E78.7 Disorders of bile acid and cholesterol metabolism
E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
E78.71 Barth syndrome
E78.72 Smith-Lemli-Opitz syndrome
E78.79 Other disorders of bile acid and cholesterol metabolism
E78.8 Other disorders of lipoprotein metabolism
E78.81 Lipoid dermatoarthritis
E78.89 Other lipoprotein metabolism disorders
E78.9 Disorder of lipoprotein metabolism, unspecified
E79 Disorders of purine and pyrimidine metabolism

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.