D80.0 vs D81
D80.0 and D81 can never be reported together. ICD-10-CM declares them mutually exclusive with an Excludes1 note, meaning the two conditions cannot occur in the same patient — so exactly one of them is correct for any given encounter.
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 (acquired (secondary)) (nonfamilial)
- Agammaglobulinemia › Bruton's X-linked (acquired (secondary)) (nonfamilial)
- Agammaglobulinemia › congenital sex-linked (acquired (secondary)) (nonfamilial)
- Agammaglobulinemia › hereditary (acquired (secondary)) (nonfamilial)
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