What is ICD-10 code E70.330?

E70.330 is the ICD-10-CM diagnosis code for Chediak-Higashi syndrome. Chediak-Higashi syndrome is a rare inherited disorder affecting immune cells and pigmentation, causing recurrent infections and abnormal bleeding due to defective white blood cells. It belongs to the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89). E70.330 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E70.330 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E70.330 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E70.330 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

What is the V28 vs V24 difference for E70.330?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E70.330 was a payment HCC under V24 (Other Significant Endocrine and Metabolic Disorders) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What should coders know when reporting E70.330?

This is a specific diagnosis code; do not use the unspecified code E70.339 if Chediak-Higashi syndrome is documented Often associated with immunodeficiency and hematologic complications; review documentation for related conditions to code comprehensively Because E70.330 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

2026 ICD-10-CM E70.330: Chediak-Higashi syndrome

ICD-10-CM Code View

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Code lookupE70.330Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E70.330

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Chediak-Higashi syndrome

Chediak-Higashi syndrome is a rare inherited disorder affecting immune cells and pigmentation, causing recurrent infections and abnormal bleeding due to defective white blood cells.

Buddy Insight

Chediak-Higashi syndrome is a rare autosomal recessive disorder caused by mutations in the LYST gene, affecting melanin production and immune cell function simultaneously.

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.194

ACA/HHS

Mapped

HCC 28

HHS-HCC 28

Varies by metal level

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.036

RXHCC

Mapped

HCC 43

Other Significant Endocrine a...

RAF 0.063

Code Book Path

Official

E70.3Albinism

E70.33Albinism with hematologic abnormality

E70.330Chediak-Higashi syndrome

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E70.330 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E70.330 in this effective period.

Related Child Codes

Official

E70.331Hermansky-Pudlak syndrome

E70.338Other albinism with hematologic abnormality

E70.339Albinism with hematologic abnormality, unspecified

Includes

Official

ICD-10-CM does not list Includes notes for E70.330 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E70.330 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E70.330 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E70.330 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E70.330 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Documentation must include the confirmed diagnosis through genetic testing or characteristic giant granules on peripheral blood smear, clinical manifestations including infections, bleeding episodes, and neurological symptoms, immunologic workup results, and current treatment status.

If the patient has undergone or is being evaluated for bone marrow transplantation, this should be documented.

Infection history and prophylactic antibiotic regimen should be noted.

MEAT Support

HCC Buddy guidance

If the patient has undergone or is being evaluated for bone marrow transplantation, this should be documented.

Infection history and prophylactic antibiotic regimen should be noted.

Audit Caution

HCC Buddy guidance

Do not confuse with Hermansky-Pudlak syndrome, which has a different genetic basis and primarily involves pulmonary fibrosis rather than immunodeficiency. Ensure the accelerated phase (hemophagocytic lymphohistiocytosis) is coded separately when present with D76.

Code all infection complications individually. This is a multi-system disorder requiring comprehensive coding of all manifestations.

Common Mistakes

HCC Buddy guidance

E70.331 (Hermansky-Pudlak syndrome) also combines albinism with hematologic abnormalities but differs in mechanism and presentation.

E70.338 (Other albinism with hematologic abnormality) covers other combined syndromes.

D71 (Functional disorders of polymorphonuclear neutrophils) may be coded additionally.

D69.1 (Qualitative platelet defects) captures the bleeding component.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E70.330 an HCC code?

Yes. E70.330 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code: E70.330
Description: Chediak-Higashi syndrome
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders

0.194

ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders

0.036

RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders

0.063

Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work E70.330 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E70.330

For E70.330to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E70.330 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E70.330 is the ICD-10-CM diagnosis code for chediak-higashi syndrome. Chediak-Higashi syndrome is a rare inherited disorder affecting immune cells and pigmentation, causing recurrent infections and abnormal bleeding due to defective white blood cells. E70.330 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E70.330 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

This is a specific diagnosis code; do not use the unspecified code E70.339 if Chediak-Higashi syndrome is documented. Because E70.330 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E70.330 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•This is a specific diagnosis code; do not use the unspecified code E70.339 if Chediak-Higashi syndrome is documented
•Often associated with immunodeficiency and hematologic complications; review documentation for related conditions to code comprehensively

Clinical Significance

Chediak-Higashi syndrome is a rare autosomal recessive disorder caused by mutations in the LYST gene, affecting melanin production and immune cell function simultaneously. Patients present with partial oculocutaneous albinism, recurrent pyogenic infections due to defective neutrophil and natural killer cell function, a bleeding tendency from platelet storage pool deficiency, and progressive neurological deterioration. The accelerated phase (hemophagocytic lymphohistiocytosis) is frequently fatal without bone marrow transplantation.

Documentation Requirements

✓Documentation must include the confirmed diagnosis through genetic testing or characteristic giant granules on peripheral blood smear, clinical manifestations including infections, bleeding episodes, and neurological symptoms, immunologic workup results, and current treatment status.
✓If the patient has undergone or is being evaluated for bone marrow transplantation, this should be documented.
✓Infection history and prophylactic antibiotic regimen should be noted.

Commonly Confused Codes

•E70.331 (Hermansky-Pudlak syndrome) also combines albinism with hematologic abnormalities but differs in mechanism and presentation.
•E70.338 (Other albinism with hematologic abnormality) covers other combined syndromes.
•D71 (Functional disorders of polymorphonuclear neutrophils) may be coded additionally.
•D69.1 (Qualitative platelet defects) captures the bleeding component.
•D76.1 (Hemophagocytic lymphohistiocytosis) codes the accelerated phase.