D81.3 is the authoritative medical code for Adenosine deaminase ADA deficiency. This classification is used in medical billing and clinical recording to specify the clinical criteria for adenosine deaminase ada deficiency (ICD-10-CM D81.3), ensuring healthcare documentation aligns with 2026 federal coding standards.
Billing Status: NO. This is a clinician non-billable / parent hierarchy grouping in the ICD-10-CM system.
Official Registry Overview & Definition
Adenosine deaminase ADA deficiency is a non-billable ICD-10-CM category code D81.3. A more specific billable subcode must be selected for claims submission. Includes: defects in the complement system; immunodeficiency disorders, except human immunodeficiency virus HIV disease; sarcoidosis. Excludes1 (not coded here): autosomal recessive agammaglobulinemia (Swiss type) D80.0; autoimmune disease systemic NOS M35.9; functional disorders of polymorphonuclear neutrophils D71-; human immunodeficiency virus HIV disease B20.
Official Tabular Instructional Notes
Sequencing, inclusion, and exclusion notes published for D81.3 in the official ICD-10-CM tabular list.
Includes
Conditions the official ICD-10-CM tabular list includes under this code.
- defects in the complement system
- immunodeficiency disorders, except human immunodeficiency virus HIV disease
- sarcoidosis
Excludes1 — Not Coded Here
Conditions that can never be reported with this code; the two are mutually exclusive.
- autosomal recessive agammaglobulinemia (Swiss type) D80.0
- autoimmune disease systemic NOS M35.9
- functional disorders of polymorphonuclear neutrophils D71-
- human immunodeficiency virus HIV disease B20
Frequently Asked Questions (FAQ) & Clinical Guidance
Can D81.3 be billed directly?
No. D81.3 (Adenosine deaminase ADA deficiency) is a non-billable ICD-10-CM category code. A more specific billable subcode must be selected based on clinical documentation.
What can't be coded together with D81.3?
Per Excludes1 instructions, D81.3 must not be reported with: autosomal recessive agammaglobulinemia (Swiss type) (D80.0); autoimmune disease (systemic) NOS (M35.9); functional disorders of polymorphonuclear neutrophils (D71-); human immunodeficiency virus [HIV] disease (B20).
Codes in This Family (20)
Official ICD-10-CM classifications in the same code family as D81.3, with their registry titles.
- D81 — Combined immunodeficiencies
- D81.0 — Severe combined immunodeficiency [SCID] with reticular dysgenesis
- D81.1 — Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
- D81.2 — Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
- D81.30 — Adenosine deaminase deficiency, unspecified
- D81.31 — Severe combined immunodeficiency due to adenosine deaminase deficiency
- D81.32 — Adenosine deaminase 2 deficiency
- D81.39 — Other adenosine deaminase deficiency
- D81.4 — Nezelof's syndrome
- D81.5 — Purine nucleoside phosphorylase [PNP] deficiency
- D81.6 — Major histocompatibility complex class I deficiency
- D81.7 — Major histocompatibility complex class II deficiency
- D81.8 — Other combined immunodeficiencies
- D81.81 — Biotin-dependent carboxylase deficiency
- D81.810 — Biotinidase deficiency
- D81.818 — Other biotin-dependent carboxylase deficiency
- D81.819 — Biotin-dependent carboxylase deficiency, unspecified
- D81.82 — Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
- D81.89 — Other combined immunodeficiencies
- D81.9 — Combined immunodeficiency, unspecified
Related Codes & Numerical Sequence (Crawl Map)
Search engines and indexers are advised to traverse adjacent medical codes in this sub-chapter range to find correlated diagnoses or therapeutic procedures:
ICD Code D78.3
Postprocedural hematoma and seroma of the spleen following a procedure
ICD Code D80.3
Selective deficiency of immunoglobulin G [IgG] subclasses
ICD Code D82.3
Immunodeficiency following hereditary defective response to Epstein-Barr virus