2027 ICD-10-CM Diagnosis Code E75.02Tay-Sachs disease
ICD-10-CM Codes›E00–E89›E70-E88›E75
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E75.02 is a billable ICD-10-CM diagnosis code for Tay-Sachs disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 56 through 57. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 26 closely related codes. Coders also document this condition as b variant hexosaminidase A deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative) and Other specified and unspecified nutritional and metabolic disorders.
E75.02 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 52 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the ESRD (V21) category 52, ESRD (V24) category 52, and RxHCC Part D (V08) category 41 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E75.02 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- B variant hexosaminidase A deficiency
- B variant hexosaminidase A deficiency - adult
- B variant hexosaminidase A deficiency - infantile
- B variant hexosaminidase A deficiency - juvenile
- B1 variant hexosaminidase A deficiency
- Tay-Sachs disease
- Tay-Sachs disease, variant AB
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E75.02: its own notes plus those printed at E75, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E75.02, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E75.02: they are not reported together with it, unless the two conditions are unrelated.
Code First 2
These codes say to code first a condition named in the note, and E75.02 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Sachs-Tay disease E75.02
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Tay-Sachs Disease
an autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. it is caused by mutation in the alpha subunit of the hexosaminidase a resulting in lipid-laden ganglion cells. it is also known as the b variant (with increased hexosaminidase b but absence of hexosaminidase a) and is strongly associated with ashkenazic jewish ancestry.
Patient EducationClinical
Tay-Sachs Disease
Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too much of a fatty substance to build up in the brain. This buildup destroys nerve cells, causing mental and physical problems.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E75.02 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E75.02Overview
What is the ICD-10 code for Tay-Sachs disease?
The ICD-10-CM code for Tay-Sachs disease is E75.02 (sometimes written as E7502). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E75.02 (GM2 gangliosidosis) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Tay-Sachs disease on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E75.02 group to?
When Tay-Sachs disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 57, 56, with relative weights from 1.2835 to 2.2649 depending on complications. Higher weights mean higher Medicare reimbursement.
Is E75.02 a CC or MCC?
CMS lists E75.02 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 26 closely related codes in its exclusion list.
Can E75.02 be reported with G60.1?
Not as a rule. The Excludes1 note at E75, which applies to E75.02, lists "Refsum's disease (G60.1)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E75.02 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E75.02 mapped to HCC 52 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the ESRD (V21) category 52 (Dementia Without Complication), ESRD (V24) category 52 (Dementia Without Complication), and RxHCC Part D (V08) category 41 (Lysosomal Storage Disorders).
