ICD-10-CM 2027
ICD-10 code for Agammaglobulinemia)
From the official ICD-10-CM alphabetic index entry “Agammaglobulinemia (acquired (secondary)) (nonfamilial)”. Page updated September 29, 2026.
About coding Agammaglobulinemia)
The ICD-10-CM code for Agammaglobulinemia) is D80.1 (Nonfamilial hypogammaglobulinemia). The official index lists 10 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on D80.1.
Within D80 (immunodeficiency with predominantly antibody defects), D80.1 is specifically for nonfamilial hypogammaglobulinemia. Related codes cover hereditary hypogammaglobulinemia (D80.0), selective deficiency of immunoglobulin A [IgA] (D80.2), selective deficiency of immunoglobulin G [IgG] subclasses (D80.3), selective deficiency of immunoglobulin M [IgM] (D80.4), immunodeficiency with increased immunoglobulin M [IgM] (D80.5), antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia (D80.6), transient hypogammaglobulinemia of infancy (D80.7), other immunodeficiencies with predominantly antibody defects (D80.8) and 1 more.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 814 (Reticuloendothelial and Immunity Disorders with MCC, relative weight 2.0729), DRG 815 (Reticuloendothelial and Immunity Disorders with CC, relative weight 1.0444) and DRG 816 (Reticuloendothelial and Immunity Disorders without CC/MCC, relative weight 0.7381), in MDC 16 (Diseases and Disorders of the Blood and Blood Forming Organs and Immunological Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific Agammaglobulinemia) codes (official index)
- with
- immunoglobulin-bearing B-lymphocytes D80.1 — Nonfamilial hypogammaglobulinemia
- lymphopenia D81.9 — Combined immunodeficiency, unspecified
- autosomal recessive (Swiss type) D80.0 — Hereditary hypogammaglobulinemia
- Bruton's X-linked D80.0 — Hereditary hypogammaglobulinemia
- common variable (CVAgamma) D80.1 — Nonfamilial hypogammaglobulinemia
- congenital sex-linked D80.0 — Hereditary hypogammaglobulinemia
- hereditary D80.0 — Hereditary hypogammaglobulinemia
- lymphopenic D81.9 — Combined immunodeficiency, unspecified
- Swiss type (autosomal recessive) D80.0 — Hereditary hypogammaglobulinemia
- X-linked (with growth hormone deficiency) (Bruton) D80.0 — Hereditary hypogammaglobulinemia
Common questions about Agammaglobulinemia) ICD-10 codes
What is the ICD-10 code for Agammaglobulinemia)?
- D80.1 — Nonfamilial hypogammaglobulinemia. More specific codes apply when the documentation supports them: D80.1, D81.9, D80.0, D80.0 and others below.
What is the ICD-10 code for autosomal recessive agammaglobulinemia)?
- D80.0 — Hereditary hypogammaglobulinemia.
What is the ICD-10 code for bruton's x-linked agammaglobulinemia)?
- D80.0 — Hereditary hypogammaglobulinemia.
What is the ICD-10 code for common variable agammaglobulinemia)?
- D80.1 — Nonfamilial hypogammaglobulinemia.
What is the ICD-10 code for congenital sex-linked agammaglobulinemia)?
- D80.0 — Hereditary hypogammaglobulinemia.
What is the ICD-10 code for hereditary agammaglobulinemia)?
- D80.0 — Hereditary hypogammaglobulinemia.
What is the ICD-10 code for lymphopenic agammaglobulinemia)?
- D81.9 — Combined immunodeficiency, unspecified.
Is D80.1 billable?
- Yes. D80.1 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can D80.1 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict D80.1 as a principal diagnosis.
Is D80.1 a CC or MCC?
- D80.1 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.