E78.6
BillableLipoprotein deficiency
HCC Category Mapping
RxHCCHCC 47 — Disorders of Fatty-Acid and Lipid Metabolism
0.000What This Code Means
A rare condition where the body lacks or has deficient levels of certain lipoproteins needed to transport fats in the blood.
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 deficiencyE78.89 — Other lipoprotein metabolism disorders: for lipoprotein abnormalities not classified as deficiencyE78.9 — Disorder of lipoprotein metabolism, unspecified: too nonspecific when deficiency is confirmedK90.49 — Malabsorption due to intolerance, not elsewhere classified: fat malabsorption in lipoprotein deficiency is metabolic, not intestinal
Code Hierarchy
└E78Disorders of lipoprotein metabolism and other lipidemias└E78.6Lipoprotein deficiency
└E78.6Lipoprotein deficiency