D80.0 Hereditary hypogammaglobulinemia

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applicable to

• Autosomal recessive agammaglobulinemia (Swiss type)
• X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 115 — Specified Immunodeficiencies and White Blood Cell Disorders
• CMS-HCC V22: HCC 47 — Disorders of Immunity
• RxHCC V08: HCC 99 — Immune Disorders
• ESRD V24: HCC 47

MS-DRG index

Defines the logic of DRG(s) 814-816 in MDC 16.

Approximate ICD-9-CM (GEMs)

• 279.04 Congenital hypogammaglobulinemia

Code annotations containing back-references to D80.0

Back-references to D80.0

• Type 1 Excludes: D81 Combined immunodeficiencies ("autosomal recessive agammaglobulinemia (Swiss type) (D80.0)")

Back-references to D80

• Type 1 Excludes: D72 Other disorders of white blood cells ("immunity disorders (D80-D89)")
• Type 1 Excludes: D84.81 Immunodeficiency due to conditions classified elsewhere ("certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)")
• Code First: M36.8 Systemic disorders of connective tissue in other diseases classified elsewhere ("hypogammaglobulinemia (D80.-)")

Diagnosis Index entries for D80.0

• Absence (of) (organ or part) (complete or partial) › gamma globulin in blood › hereditary
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › autosomal recessive (Swiss type)
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › Bruton's X-linked
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › congenital sex-linked
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › hereditary
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › Swiss type (autosomal recessive)
• Agammaglobulinemia (acquired (secondary)) (nonfamilial) › X-linked (with growth hormone deficiency) (Bruton)
• Bruton's X-linked agammaglobulinemia
• Deficiency, deficient › gammaglobulin in blood › hereditary
• Hypogammaglobulinemia › hereditary
• Immunodeficiency › autosomal recessive, Swiss type
• Syndrome › antibody deficiency › agammaglobulinemic › hereditary
• Syndrome › antibody deficiency › congenital
• Syndrome › antibody deficiency › hypogammaglobulinemic › hereditary

Nearby codes

• D80 Immunodeficiency with predominantly antibody defects
• D80.0 Hereditary hypogammaglobulinemia (this code)
• D80.1 Nonfamilial hypogammaglobulinemia
• D80.2 Selective deficiency of immunoglobulin A [IgA]
• D80.3 Selective deficiency of immunoglobulin G [IgG] subclasses
• D80.4 Selective deficiency of immunoglobulin M [IgM]
• D80.5 Immunodeficiency with increased immunoglobulin M [IgM]