ICD-10/D81.0

D81.0 ICD 2026 Code: Severe combined immunodeficiency SCID with reticular dysgenesis

D81.0 is the authoritative medical code for Severe combined immunodeficiency SCID with reticular dysgenesis. This classification is used in medical billing and clinical recording to specify the clinical criteria for severe combined immunodeficiency scid with reticular dysgenesis (ICD-10-CM D81.0), ensuring healthcare documentation aligns with 2026 federal coding standards.

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Official Registry Overview & Definition

Severe combined immunodeficiency SCID with reticular dysgenesis is a billable ICD-10-CM diagnosis code D81.0. 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 D81.0 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

What can't be coded together with D81.0?

Per Excludes1 instructions, D81.0 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 (13)

Official ICD-10-CM classifications in the same code family as D81.0, with their registry titles.

  • D81 — Combined immunodeficiencies
  • 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
  • D81.3 — Adenosine deaminase [ADA] 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
  • D81.810 — Biotinidase deficiency
  • D81.818 — Other biotin-dependent carboxylase deficiency
  • D81.819 — Biotin-dependent carboxylase deficiency, unspecified
  • D81.9 — Combined immunodeficiency, unspecified

Indexed Clinical Terms (1)

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.

  • Immunodeficiency, combined, severe (SCID), with, reticular dysgenesis

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 D78.0 Intraoperative hemorrhage and hematoma of the spleen complicating a procedure
ICD Code D80.0 Hereditary hypogammaglobulinemia
ICD Code D82.0 Wiskott-Aldrich syndrome
ICD Code D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
ICD Code D84.0 Lymphocyte function antigen-1 [LFA-1] defect
ICD Code D86.0 Sarcoidosis of lung
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