2026 ICD-10-CM Diagnosis Code E70.21Tyrosinemia

ICD-10-CM Codes›E00–E89›E70-E88›E70

ICD-10-CM E70.21
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E70.21 is a billable ICD-10-CM diagnosis code for tyrosinemia. 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
E70.21
Billable Status
Yes — Valid for Submission
Code Describes
Tyrosinemia
Short Description
Tyrosinemia
Same as the full description in the CMS dataset.
Parent Code
E70.2
Disorders of tyrosine metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE70Disorders of aromatic amino-acid metabolism
This CodeE70.21Tyrosinemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 4-Hydroxyphenylpyruvate dioxygenase deficiency
  • Classical phenylketonuria
  • Clinical manifestation of enzyme deficiency
  • Deficiency of fumarylacetoacetase
  • Fumarylacetoacetase deficiency, chronic type
  • Hereditary hypertyrosinemia
  • Hypertyrosinemia
  • Persistent hyperphenylalaninemia
  • Persistent hyperphenylalaninemia AND tyrosinemia
  • Phenylketonuria
  • Tyrosinemia type 1
  • Tyrosinemia type 2
  • Tyrosinemia type III
  • Tyrosinosis

Tabular List NotesGuidance

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

Inclusion Terms

  • Hypertyrosinemia

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.

    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • aromatic
            • tyrosine
              • hypertyrosinemia
    • Hypertyrosinemia
    • Tyrosinemia
    • Tyrosinosis

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

  • Tyrosinemia

    an autosomal recessive inherited metabolic disorder caused by mutations in the fah, hpd, and tat genes. it is characterized by deficiency of one of the enzymes that are involved in the metabolism of tyrosine. it results in elevated blood tyrosine levels and accumulation of tyrosine and its byproducts in the liver, kidney, nervous system and other organs.

  • Tyrosinemia Type I|Type I Tyrosinemia

    tyrosinemia caused by mutations in the fah gene. it is characterized by deficiency of the enzyme fumarylacetoacetate hydrolase. it is the most severe form of tyrosinemia. signs and symptoms appear early in life and include failure to thrive, vomiting, diarrhea, jaundice, and bleeding tendency. it may result in liver and kidney failure. patients with this type of tyrosinemia may also have a predisposition for hepatocellular carcinoma.

  • Tyrosinemia Type II

    tyrosinemia caused by mutation(s) in the tat gene, encoding tyrosine aminotransferase. the inheritance is autosomal recessive.

Patient EducationClinical

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E70.21 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
270.2 Arom amin-acid metab NEC
Approximate The match is approximate rather than exact.

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 E70.21Overview

Is E70.21 (Disorders of tyrosine metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report tyrosinemia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E70.21?

Under the General Equivalence Mappings, tyrosinemia converts to ICD-9-CM 270.2 (arom amin-acid metab NEC). The mapping is approximate, so confirm the match fits the documentation.

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.