What is ICD-10 code E75.240?

E75.240 is the ICD-10-CM diagnosis code for Niemann-Pick disease type A. Niemann-Pick disease type A is a severe inherited metabolic disorder where the body cannot break down cholesterol and other fatty substances, causing rapid neurological deterioration, organ enlargement, and typically fatal outcomes in early childhood. E75.240 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.240 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. E75.240 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. E75.240 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 E75.240?

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. E75.240 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 E75.240?

Type A is the most severe form with early neurological involvement; ensure accurate type specification in documentation Code associated complications such as hepatosplenomegaly, developmental regression, or seizures separately Because E75.240 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.

E75.240: Niemann-Pick disease type A — ICD-10 | Other Significant Endocrine and Metabolic

ICD-10-CM Code View

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Code lookupE75.240Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E75.240

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

Niemann-Pick disease type A

Niemann-Pick disease type A is a severe inherited metabolic disorder where the body cannot break down cholesterol and other fatty substances, causing rapid neurological deterioration, organ enlargement, and typically fatal outcomes in early childhood.

Buddy Insight

Niemann-Pick disease type A is the most severe form of acid sphingomyelinase deficiency, causing massive sphingomyelin accumulation in the brain and visceral organs.

CMS-HCC V28

Not risk-adjusted for this model/period.

RAF 0

CMS-HCC V24

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.230

ACA/HHS

Not risk-adjusted for this model/period.

RAF 0

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.0

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

RAF 0.0

Code Trumping

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Code Book Path

Official

E75.2Other sphingolipidosis

E75.24Niemann-Pick disease

E75.240Niemann-Pick disease type A

Inclusion Terms

Official

Acid sphingomyelinase deficiency type A (ASMD type A)
Infantile neurovisceral acid sphingomyelinase deficiency

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.240 in this effective period.

Related Child Codes

Official

E75.241Niemann-Pick disease type B

E75.242Niemann-Pick disease type C

E75.243Niemann-Pick disease type D

E75.244Niemann-Pick disease type A/B

E75.248Other Niemann-Pick disease

Includes

Official

ICD-10-CM does not list Includes notes for E75.240 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E75.240 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.240 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.240 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.240 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Confirmed diagnosis of Niemann-Pick disease type A

Acid sphingomyelinase enzyme assay showing severe deficiency

SMPD1 gene mutation analysis confirming type A mutations

Documentation of hepatosplenomegaly on exam or imaging

MEAT Support

HCC Buddy guidance

Confirmed diagnosis of Niemann-Pick disease type A

Acid sphingomyelinase enzyme assay showing severe deficiency

SMPD1 gene mutation analysis confirming type A mutations

Documentation of hepatosplenomegaly on exam or imaging

Audit Caution

HCC Buddy guidance

Confusing Niemann-Pick type A with type C — they involve different genes and enzymes despite the same disease name

Using the unspecified Niemann-Pick code when the type has been confirmed

Not recognizing that both Tay-Sachs and Niemann-Pick type A can present with cherry-red macular spots

Failing to code organ-specific complications as additional diagnoses

Common Mistakes

HCC Buddy guidance

E75.241 — Niemann-Pick disease type B: visceral involvement without severe neurological disease

E75.244 — Niemann-Pick disease type A/B: intermediate phenotype with features of both

E75.242 — Niemann-Pick disease type C: different gene and enzyme (NPC1/NPC2), not sphingomyelinase

E75.249 — Niemann-Pick disease, unspecified: do not use when type A is confirmed

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 E75.240 an HCC code?

Yes. E75.240 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 41, Lysosomal Storage Disorders

0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. 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.

MEAT Criteria for E75.240

For E75.240to 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 E75.240 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.240 is the ICD-10-CM diagnosis code for niemann-pick disease type a. Niemann-Pick disease type A is a severe inherited metabolic disorder where the body cannot break down cholesterol and other fatty substances, causing rapid neurological deterioration, organ enlargement, and typically fatal outcomes in early childhood. E75.240 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, E75.240 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. 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.

Type A is the most severe form with early neurological involvement; ensure accurate type specification in documentation. Because E75.240 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 E75.240 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Type A is the most severe form with early neurological involvement; ensure accurate type specification in documentation
•Code associated complications such as hepatosplenomegaly, developmental regression, or seizures separately

Clinical Significance

Niemann-Pick disease type A is the most severe form of acid sphingomyelinase deficiency, causing massive sphingomyelin accumulation in the brain and visceral organs. Infants develop hepatosplenomegaly, failure to thrive, progressive neurological deterioration, and cherry-red macular spots. The disease is uniformly fatal by age 3. There is currently no disease-modifying treatment, making accurate coding important for palliative care resource allocation.

Documentation Requirements

✓Confirmed diagnosis of Niemann-Pick disease type A
✓Acid sphingomyelinase enzyme assay showing severe deficiency
✓SMPD1 gene mutation analysis confirming type A mutations
✓Documentation of hepatosplenomegaly on exam or imaging
✓Neurological assessment documenting developmental regression
✓Ophthalmologic findings including cherry-red macular spot if present

Commonly Confused Codes

•E75.241: Niemann-Pick disease type B: visceral involvement without severe neurological disease
•E75.244: Niemann-Pick disease type A/B: intermediate phenotype with features of both
•E75.242: Niemann-Pick disease type C: different gene and enzyme (NPC1/NPC2), not sphingomyelinase
•E75.249: Niemann-Pick disease, unspecified: do not use when type A is confirmed
•E75.02: Tay-Sachs disease: also has cherry-red spot but different enzyme deficiency