Excludes1 pair

D80.0 vs D81-

D80.0 and D81 are not coded together. ICD-10-CM declares them mutually exclusive with an Excludes1 note — the two conditions are not expected to occur together, so exactly one of them is normally correct for a given encounter. The Official Guidelines carve out one exception: if the two conditions are clearly unrelated to each other, both may be reported.

The official note, declared on D81
autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
D80.0

Hereditary hypogammaglobulinemia

Billable
Chapter 3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Section
D80-D89
Use when the record says
  • Autosomal recessive agammaglobulinemia (Swiss type)
  • X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)
Indexed under
  • Bruton's X-linked agammaglobulinemia
  • Agammaglobulinemia › autosomal recessive (Swiss type)
  • Agammaglobulinemia › Bruton's X-linked
  • Agammaglobulinemia › congenital sex-linked
  • Agammaglobulinemia › hereditary
D81

Combined immunodeficiencies

Not billable
Chapter 3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Section
D80-D89

Excludes1 is not always absolute: where two conditions covered by the note are genuinely unrelated, CMS guidance permits reporting both. Document the reasoning rather than assuming it. More on Excludes1 vs Excludes2 · FY2027