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FY2027 ICD-10-CM reference

E88.43: Disorders of mitochondrial tRNA synthetases

By the HCC Buddy Coding Team

The official ICD-10-CM descriptor is Disorders of mitochondrial tRNA synthetases. The FY2027 annual edition starts October 1, 2026. Check the edition that applies to the encounter or inpatient discharge, including any April update.

Billable code. Verified against the official FY2027 order file.

CMS-HCC V28

No V28 HCC for 2026 dates of service (CMS 2027 Initial ICD-10-CM Mappings)

CMS-HCC V24

Not used for payment year 2027 Medicare Advantage payment

ESRD

  • HCC 23: Other Significant Endocrine and Metabolic Disorders
  • CMS lists the same category for every age band in its model mapping

No RAF is shown: it depends on the member. Mapping: PY2026 CMS mapping; CMS 2027 initial file unchanged.

PACE

Source verification pending

RxHCC

  • HCC 43: Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders

Coefficient pending: payment year 2027 coefficients for this model are not yet verified, so no RAF is shown. Mapping: PY2026 CMS mapping; CMS 2027 initial file unchanged.

HHS-HCC

  • HCC 28: HHS-HCC 28
  • This code has a CMS age requirement: applies when age < 21

No RAF is shown: it depends on the member. Mapping: HHS-HCC 2026 benefit year.

FY2027 Tabular instructions

Instructions below include applicable chapter, section and parent-code notes. Each note identifies where it appears in the official source.

Instructional note

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: E00-E89 (chapter, inherited).

Excludes1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Source: E00-E89 (chapter, inherited).

Excludes1

androgen insensitivity syndrome (E34.5-)

Source: E70-E88 (section, inherited).

Excludes1

congenital adrenal hyperplasia (E25.0)

Source: E70-E88 (section, inherited).

Excludes1

hemolytic anemias attributable to enzyme disorders (D55.-)

Source: E70-E88 (section, inherited).

Excludes1

Marfan syndrome (Q87.4-)

Source: E70-E88 (section, inherited).

Excludes1

5-alpha-reductase deficiency (E29.1)

Source: E70-E88 (section, inherited).

Excludes2

Ehlers-Danlos syndromes (Q79.6-)

Source: E70-E88 (section, inherited).

Instructional note

codes for associated conditions

Source: E88 (category, inherited).

Excludes1

histiocytosis X (chronic) (C96.6)

Source: E88 (category, inherited).

Excludes1

disorders of pyruvate metabolism (E74.4)

Source: E88.4 (self, inherited).

Excludes1

Kearns-Sayre syndrome (H49.81)

Source: E88.4 (self, inherited).

Excludes1

Leber's disease (H47.22)

Source: E88.4 (self, inherited).

Excludes1

Leigh's encephalopathy (G31.82)

Source: E88.4 (self, inherited).

Excludes1

Mitochondrial myopathy, NEC (G71.3)

Source: E88.4 (self, inherited).

Excludes1

Reye's syndrome (G93.7)

Source: E88.4 (self, inherited).

Instructional note

ARS2-related mitochondrial disorders

Source: E88.43 (self, direct).

Instructional note

LBSL

Source: E88.43 (self, direct).

Instructional note

Leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation

Source: E88.43 (self, direct).

Instructional note

Leukoencephalopathy with thalamus - brainstem involvement - high lactate

Source: E88.43 (self, direct).

Instructional note

LTBL

Source: E88.43 (self, direct).

Instructional note

Mitochondrial aminoacyl-tRNA synthetase disorders

Source: E88.43 (self, direct).

Code also

, if applicable, associated condition such as:

Source: E88.43 (self, direct).

Code also

leukoencephalopathy (G93.49)

Source: E88.43 (self, direct).

Source: CMS FY2027 Tabular List and Alphabetic Index.

Open Tabular instructions and code details

Compare the FY2027 additions, removals and billable-status changes

What does E88.43 mean in FY2027?

The official ICD-10-CM descriptor is Disorders of mitochondrial tRNA synthetases. The FY2027 annual edition starts October 1, 2026. Check the edition that applies to the encounter or inpatient discharge, including any April update.

Is E88.43 billable in FY2027?

The FY2027 order file marks E88.43 as billable. Check the complete Tabular instructions and documentation before assigning it.

Does E88.43 map to an HCC?

E88.43 has no CMS-HCC V28 category in the CMS 2027 Initial ICD-10-CM Mappings (payment year 2027, 2026 dates of service). Under the PY2026 CMS mapping; CMS 2027 initial file unchanged: ESRD HCC 23 (CMS lists the same category for every age band in its model mapping); RxHCC HCC 43. Coefficients for these models are pending verification for payment year 2027. HHS-HCC: HCC 28 (This code has a CMS age requirement: applies when age < 21) under the HHS-HCC 2026 benefit year mapping (no RAF; HHS-HCC weights vary by metal level). CMS-HCC V24: not used for payment year 2027 Medicare Advantage payment (CMS CY 2027 Rate Announcement). The PACE mapping is still pending verification for FY2027.

Source: CMS FY2027 ICD-10-CM order file. Payment model: CMS CY 2027 Rate Announcement. Read the FY2027 Official Guidelines.

Review the FY2026 April edition