ICD-10-CM 2027
ICD-10 code for Chédiak-Higashi syndrome
From the official ICD-10-CM alphabetic index entry “Chédiak-Higashi (-Steinbrinck) syndrome (congenital gigantism of peroxidase granules)”. Page updated October 2, 2026.
About coding Chédiak-Higashi syndrome
The ICD-10-CM code for Chédiak-Higashi syndrome is E70.330 (Chediak-Higashi syndrome).
Within E70.33 (albinism with hematologic abnormality), E70.330 is specifically for Chediak-Higashi syndrome. Related codes cover Hermansky-Pudlak syndrome (E70.331), other albinism with hematologic abnormality (E70.338) and unspecified (E70.339).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Chédiak-Higashi syndrome ICD-10 codes
What is the ICD-10 code for Chédiak-Higashi syndrome?
- E70.330 — Chediak-Higashi syndrome.
Is E70.330 billable?
- Yes. E70.330 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E70.330 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E70.330 as a principal diagnosis.
Is E70.330 a CC or MCC?
- E70.330 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.