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  6. 2026 ICD-10-CM Code E78.010

2026 ICD-10-CM Diagnosis Code E78.010

Homozygous familial hypercholesterolemia [HoFH]

ICD-10-CM Code:
E78.010
ICD-10 Code for:
Homozygous familial hypercholesterolemia [HoFH]
Is Billable?
Yes - Valid for Submission
Code Navigator:

E78.010 is a billable diagnosis code used to specify a medical diagnosis of homozygous familial hypercholesterolemia [hofh]. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2025 through September 30, 2026.

Code Classification

  • Endocrine, nutritional and metabolic diseases
    E00–E89
    • Metabolic disorders
      E70-E88
      • Disorders of lipoprotein metabolism and other lipidemias
        E78

Approximate Synonyms

The following list of clinical terms are approximate synonyms, alternative descriptions, or common phrases that might be used by patients, healthcare providers, or medical coders to describe the same condition. These synonyms and related diagnosis terms are often used when searching for an ICD-10 code, especially when the exact medical terminology is unclear. Whether you're looking for lay terms, similar diagnosis names, or common language alternatives, this list can help guide you to the correct ICD-10 classification.

  • Familial hypercholesterolemia
  • Familial hypercholesterolemia
  • Familial hypercholesterolemia - homozygous
  • Familial hypercholesterolemia - homozygous
  • Familial hypercholesterolemia due to homozygous LDL receptor mutation

Clinical Information

  • Homozygous Familial Hypercholesterolemia

    a rare inherited genetic disorder, one form of hyperlipoproteinemia type ii, characterized by high level of low-density lipoprotein (ldl) which if not treated could elevate the chance of heart attack at an early age.

New 2026 ICD-10-CM Code

E78.010 is new to ICD-10-CM code set for the FY 2026, effective October 1, 2025. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2025. This is a new and revised code for the FY 2026 (October 1, 2025 - September 30, 2026).

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Index of External Cause of Injuries

References found for this diagnosis code in the External Cause of Injuries Index:

    • Cholesterolemia(essential) (pure)
      • familial
        • homozygous
    • HoFH(homozygous familial hypercholesterolemia)
    • Hypercholesterolemia(essential) (primary) (pure)
      • familial
        • homozygous [HoFH]

Replacement Code

E78010 replaces the following previously assigned ICD-10-CM code(s):

  • E78.01 - Familial hypercholesterolemia

Code History

  • FY 2026 - Code Added, effective from 10/1/2025 through 9/30/2026