What is ICD-10 code E75.241?

E75.241 is the ICD-10-CM diagnosis code for Niemann-Pick disease type B. Niemann-Pick disease type B is an inherited metabolic disorder where the body cannot break down cholesterol and fatty substances, causing organ enlargement and some neurological problems, but with slower progression and longer survival than type A. E75.241 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.241 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. E75.241 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. E75.241 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 E75.241?

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. E75.241 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 E75.241?

Type B has visceral involvement without severe neurological manifestations; document presence or absence of neurological symptoms Code organ-specific complications such as hepatosplenomegaly, lung disease, or bone involvement separately Because E75.241 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.

E75.241: Niemann-Pick disease type B — ICD-10

ICD-10-CM Code View

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Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.

Code lookupE75.241Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E75.241

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

Niemann-Pick disease type B

Niemann-Pick disease type B is an inherited metabolic disorder where the body cannot break down cholesterol and fatty substances, causing organ enlargement and some neurological problems, but with slower progression and longer survival than type A.

Buddy Insight

Niemann-Pick disease type B is caused by partial acid sphingomyelinase deficiency, resulting in visceral sphingomyelin accumulation without significant neurological involvement.

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

E75.241Niemann-Pick disease type B

Inclusion Terms

Official

Acid sphingomyelinase deficiency type B (ASMD type B)
Chronic visceral acid sphingomyelinase deficiency

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.241 in this effective period.

Related Child Codes

Official

E75.240Niemann-Pick disease type A

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 E75.241 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E75.241 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.241 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.241 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.241 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Confirmed diagnosis of Niemann-Pick disease type B

Acid sphingomyelinase enzyme assay showing partial deficiency

SMPD1 gene mutation analysis confirming type B mutations

Hepatosplenomegaly assessment with imaging

MEAT Support

HCC Buddy guidance

Confirmed diagnosis of Niemann-Pick disease type B

Acid sphingomyelinase enzyme assay showing partial deficiency

SMPD1 gene mutation analysis confirming type B mutations

Hepatosplenomegaly assessment with imaging

Audit Caution

HCC Buddy guidance

Confusing type B with type A — type B lacks significant neurological involvement and has a much better prognosis

Not recognizing that interstitial lung disease is a key feature of type B that should be coded separately

Using the unspecified code when the type has been confirmed by enzyme assay or genetic testing

Missing the thrombocytopenia and hyperlipidemia as codable complications

Common Mistakes

HCC Buddy guidance

E75.240 — Niemann-Pick disease type A: severe neuronopathic form, usually fatal in infancy

E75.244 — Niemann-Pick disease type A/B: intermediate phenotype

E75.242 — Niemann-Pick disease type C: different enzyme pathway entirely

E75.249 — Niemann-Pick disease, unspecified: do not use when type B 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 E75.241 an HCC code?

Yes. E75.241 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 E75.241

For E75.241to 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 E75.241 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

E75.241 is the ICD-10-CM diagnosis code for niemann-pick disease type b. Niemann-Pick disease type B is an inherited metabolic disorder where the body cannot break down cholesterol and fatty substances, causing organ enlargement and some neurological problems, but with slower progression and longer survival than type A. E75.241 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, E75.241 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 B has visceral involvement without severe neurological manifestations; document presence or absence of neurological symptoms. Because E75.241 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 E75.241 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Type B has visceral involvement without severe neurological manifestations; document presence or absence of neurological symptoms
•Code organ-specific complications such as hepatosplenomegaly, lung disease, or bone involvement separately

Clinical Significance

Niemann-Pick disease type B is caused by partial acid sphingomyelinase deficiency, resulting in visceral sphingomyelin accumulation without significant neurological involvement. Patients develop hepatosplenomegaly, interstitial lung disease, thrombocytopenia, and dyslipidemia, with survival into adulthood. Olipudase alfa (enzyme replacement therapy) is now available. Accurate coding supports proper risk adjustment for these patients' ongoing multi-organ management.

Documentation Requirements

✓Confirmed diagnosis of Niemann-Pick disease type B
✓Acid sphingomyelinase enzyme assay showing partial deficiency
✓SMPD1 gene mutation analysis confirming type B mutations
✓Hepatosplenomegaly assessment with imaging
✓Pulmonary function testing and chest imaging for interstitial lung disease
✓Complete blood count documenting thrombocytopenia
✓Lipid panel and treatment status including enzyme replacement therapy

Commonly Confused Codes

•E75.240: Niemann-Pick disease type A: severe neuronopathic form, usually fatal in infancy
•E75.244: Niemann-Pick disease type A/B: intermediate phenotype
•E75.242: Niemann-Pick disease type C: different enzyme pathway entirely
•E75.249: Niemann-Pick disease, unspecified: do not use when type B is confirmed
•E75.22: Gaucher disease: also causes hepatosplenomegaly but different enzyme