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.

Which HCC does E75.241 map to under V28?

E75.241 has no payable CMS-HCC V28 mapping in the final FY2026 CMS file.

What RAF reference is published for E75.241?

E75.241 has no V28 reference coefficient because the final FY2026 source verifies no payable V28 mapping.

What is the V28 vs V24 difference for E75.241?

E75.241 has verified no payable V28 mapping in the final FY2026 source. Select a historical effective period before making any V24 comparison.

What documentation supports 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 E75.241 is not a CMS-HCC V28 payment code in the verified current source, so MEAT is not the payment hook. Documentation still has to support the diagnosis if you report it.

2026 ICD-10-CM E75.241: Niemann-Pick disease type B

E75.241 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools

ICD-10-CM Code View

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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

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Historical

Not used for standard CY2026 Medicare Advantage payment. Select a historical period to review V24.

Not used for CY2026 payment

ACA/HHS

Mapped

HCC 027

HHS-HCC 027 adult, RAF varies...

Code-level coefficient reference

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

Code-level coefficient reference

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

Code-level coefficient reference

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

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E75.241 an HCC code?

E75.241 is not in the CMS-HCC V28 or V24 community payment model. E75.241 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.241 also appears in the HHS-HCC commercial risk model (HCC 027 (HHS-HCC 027 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping.

Code: E75.241
Description: Niemann-Pick disease type B
HCC (V28): No CMS-HCC V28 mapping
RAF reference coefficient: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders

Not separately weighted

RxHCCHCC 41, Lysosomal Storage Disorders

Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. 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 context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work E75.241 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT Criteria for E75.241

For E75.241 to 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.

Coder workflow notes

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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).

E75.241 has no mapping under the CMS-HCC V28 or V24 community payment models. E75.241 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.241 also appears in the HHS-HCC commercial risk model (HCC 027 (HHS-HCC 027 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.

Type B has visceral involvement without severe neurological manifestations; document presence or absence of neurological symptoms.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, 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