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E78.6 ICD-10-CM Code: Lipoprotein deficiency

ICD-10-CM Code View

HCC Buddy Code Card

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E78.6

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Lipoprotein deficiency

A rare condition where the body lacks or has deficient levels of certain lipoproteins needed to transport fats in the blood.

Buddy the Bee presenting code insight

Buddy Insight

Lipoprotein deficiency encompasses conditions like abetalipoproteinemia (Bassen-Kornzweig syndrome), hypobetalipoproteinemia, and Tangier disease, where lipoprotein levels are abnormally LOW rather than high.

CMS-HCC V28

0

0

RAF 0

CMS-HCC V24

0

0

RAF 0

ACA/HHS

0

0

RAF 0

ESRD/PACE

0

0

RAF 0

RXHCC

HCC 47

RAF 0.0

Code Trumping

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Code Book Path

Official
E78Disorders of lipoprotein metabolism and other lipidemias
E78.6Lipoprotein deficiency

Inclusion Terms

Official
  • Abetalipoproteinemia
  • Depressed HDL cholesterol
  • High-density lipoprotein deficiency
  • Hypoalphalipoproteinemia
  • Hypobetalipoproteinemia (familial)

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E78.6 in this effective period.

Related Child Codes

Official
E78.0Pure hypercholesterolemia
E78.1Pure hyperglyceridemia
E78.2Mixed hyperlipidemia
E78.3Hyperchylomicronemia
E78.4Other hyperlipidemia

Includes

Official

ICD-10-CM does not list Includes notes for E78.6 in this effective period.

Excludes 1

Official
  • sphingolipidosis (E75.0-E75.3)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E78.6 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E78.6 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E78.6 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Specific lipoprotein deficiency type documented (abetalipoproteinemia, hypobetalipoproteinemia, Tangier disease)
Confirmatory lipid testing showing absent or markedly reduced lipoproteins (apoB, HDL)
Genetic testing results if available (MTTP, APOB, ABCA1 mutations)
Clinical manifestations: fat malabsorption, neurological symptoms, retinal changes, acanthocytes on blood smear

MEAT Support

HCC Buddy guidance
Specific lipoprotein deficiency type documented (abetalipoproteinemia, hypobetalipoproteinemia, Tangier disease)
Confirmatory lipid testing showing absent or markedly reduced lipoproteins (apoB, HDL)
Genetic testing results if available (MTTP, APOB, ABCA1 mutations)
Clinical manifestations: fat malabsorption, neurological symptoms, retinal changes, acanthocytes on blood smear

Audit Caution

HCC Buddy guidance
Confusing lipoprotein deficiency (too little) with hyperlipidemia (too much) — they are opposite conditions
Not recognizing Tangier disease or abetalipoproteinemia as forms of lipoprotein deficiency
Coding only the manifestations (neuropathy, retinopathy) without the underlying metabolic disorder
Using hyperlipidemia codes for patients who actually have hypolipidemia/lipoprotein deficiency

Common Mistakes

HCC Buddy guidance
E78.00 — Pure hypercholesterolemia: ELEVATED cholesterol, opposite of lipoprotein deficiency
E78.89 — Other lipoprotein metabolism disorders: for lipoprotein abnormalities not classified as deficiency
E78.9 — Disorder of lipoprotein metabolism, unspecified: too nonspecific when deficiency is confirmed
K90.49 — Malabsorption due to intolerance, not elsewhere classified: fat malabsorption in lipoprotein deficiency is metabolic, not intestinal

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E78.6 an HCC code?

No. E78.6 is a billable ICD-10-CM code but does not map to any HCC category in V28, V24, ESRD, or RxHCC.

HCC Category Mapping

RxHCCHCC 47, Disorders of Fatty-Acid and Lipid Metabolism
0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

MEAT Criteria for E78.6

For E78.6to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E78.6 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

E78.6 is the ICD-10-CM diagnosis code for lipoprotein deficiency. A rare condition where the body lacks or has deficient levels of certain lipoproteins needed to transport fats in the blood. E78.6 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E78.6 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.

E78.6 maps only to RxHCC 47 (Disorders of Fatty-Acid and Amino-Acid Metabolism) with no RAF weight. No V24 or V28 CMS-HCC mapping. Despite being rare and clinically significant, lipoprotein deficiency does not contribute to Medicare Advantage RAF scores. Coders reviewing E78.6 should check whether additional documentation would support a more specific child code in the same hierarchy that does map to a payment HCC, capturing the correct specificity is the highest-impact RAF improvement available within accurate coding.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E78.6 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • This code represents deficiency states rather than excess; verify the diagnosis carefully
  • Document the specific lipoprotein that is deficient (HDL, apolipoprotein, etc.) in the medical record

Clinical Significance

Lipoprotein deficiency encompasses conditions like abetalipoproteinemia (Bassen-Kornzweig syndrome), hypobetalipoproteinemia, and Tangier disease, where lipoprotein levels are abnormally LOW rather than high. These rare genetic disorders can cause fat malabsorption, neuropathy, retinopathy, and acanthocytosis, requiring specialized dietary management and fat-soluble vitamin supplementation.

Documentation Requirements

  • Specific lipoprotein deficiency type documented (abetalipoproteinemia, hypobetalipoproteinemia, Tangier disease)
  • Confirmatory lipid testing showing absent or markedly reduced lipoproteins (apoB, HDL)
  • Genetic testing results if available (MTTP, APOB, ABCA1 mutations)
  • Clinical manifestations: fat malabsorption, neurological symptoms, retinal changes, acanthocytes on blood smear
  • Fat-soluble vitamin supplementation plan (vitamins A, D, E, K)
  • Dietary fat restriction or modification plan

Commonly Confused Codes

  • E78.00: Pure hypercholesterolemia: ELEVATED cholesterol, opposite of lipoprotein deficiency
  • E78.89: Other lipoprotein metabolism disorders: for lipoprotein abnormalities not classified as deficiency
  • E78.9: Disorder of lipoprotein metabolism, unspecified: too nonspecific when deficiency is confirmed
  • K90.49: Malabsorption due to intolerance, not elsewhere classified: fat malabsorption in lipoprotein deficiency is metabolic, not intestinal

Child Codes

Code Hierarchy

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