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D81.3 ICD-10-CM Code: Adenosine deaminase [ADA] deficiency

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FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Certain disorders involving the immune mechanism (D80-D89)

D81.3

Header CodeICD-10-CMOfficial ICD-10-CMCodebook guidance

Adenosine deaminase [ADA] deficiency

Adenosine deaminase [ADA] deficiency

CMS-HCC V28

0

0

RAF 0

CMS-HCC V24

0

0

RAF 0

ACA/HHS

0

0

RAF 0

ESRD/PACE

0

0

RAF 0

RXHCC

0

0

RAF 0

Code Trumping

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Code Book Path

Official
D81Combined immunodeficiencies
D81.3Adenosine deaminase [ADA] deficiency

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for D81.3 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D81.3 in this effective period.

Related Child Codes

Official
D81.30Adenosine deaminase deficiency, unspecified
D81.31Severe combined immunodeficiency due to adenosine deaminase deficiency
D81.32Adenosine deaminase 2 deficiency
D81.39Other adenosine deaminase deficiency

Includes

Official

ICD-10-CM does not list Includes notes for D81.3 in this effective period.

Excludes 1

Official
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D81.3 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D81.3 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D81.3 in this effective period.

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.

What This Code Means

D81.3 is the ICD-10-CM diagnosis code for adenosine deaminase [ada] deficiency. D81.3 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering certain disorders involving the immune mechanism (d80-d89).

Header codes like D81.3 cannot be reported on claims directly, they organize child codes that share clinical context but the actual diagnosis must be coded to the highest level of specificity supported by the documentation. Coders should look at D81.3's child codes and select the one that matches the patient's documented presentation, since payers reject header codes submitted as the primary diagnosis. For risk adjustment workflows, header codes never contribute to a Medicare Advantage member's RAF score on their own; only billable child codes that happen to map to a payment HCC affect risk-adjusted plan payments.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for D81.3 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Child Codes

Code Hierarchy

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