2026 ICD-10-CM Diagnosis Code E70.330Chediak-Higashi syndrome

ICD-10-CM CodesE00–E89E70-E88E70

ICD-10-CM E70.330
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E70.330 is a billable ICD-10-CM diagnosis code for Chediak-Higashi syndrome. 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
E70.330
Billable Status
Yes — Valid for Submission
Code Describes
Chediak-Higashi syndrome
Short Description
Chediak-Higashi syndrome
Same as the full description in the CMS dataset.
Parent Code
Albinism with hematologic abnormality

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE70Disorders of aromatic amino-acid metabolism
This CodeE70.330Chediak-Higashi syndrome

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Attenuated Chédiak-Higashi syndrome
  • Chédiak-Higashi syndrome
  • Chemotactic disorder
  • Dense body defect
  • Granulocyte granule deficiency
  • Partial albinism
  • Periodontitis exacerbated by Chédiak-Higashi syndrome

Index to Diseases and InjuriesGuidance

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Albinism, albino
      • with hematologic abnormality
        • Chédiak-Higashi syndrome
    • Anomaly, anomalous(congenital) (unspecified type)
      • Chédiak-Higashi (-Steinbrinck) (congenital gigantism of peroxidase granules)
    • Chédiak-Higashi(-Steinbrinck) syndrome (congenital gigantism of peroxidase granules)
    • Disease, diseased
      • Chédiak-Steinbrinck (-Higashi) (congenital gigantism of peroxidase granules)
    • Syndrome
      • Béquez César (-Steinbrinck-Chédiak-Higashi)
    • Syndrome
      • Chédiak-Higashi (-Steinbrinck)

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

Patient EducationClinical

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

The full article covers:

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  • How does the immune system work?
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  • What can go wrong with the immune system?

Read the full article at MedlinePlus

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Convert E70.330 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 E70.330Overview

Is E70.330 (Albinism with hematologic abnormality) a billable code?

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

What is the ICD-9 equivalent of E70.330?

Under the General Equivalence Mappings, Chediak-Higashi syndrome 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.