2026 ICD-10-CM Diagnosis Code D80.0Hereditary hypogammaglobulinemia

ICD-10-CM Codes›D50–D89›D80-D89›D80

ICD-10-CM D80.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D80.0 is a billable ICD-10-CM diagnosis code for hereditary hypogammaglobulinemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 814 through 816. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

Code Identity

ICD-10-CM Code
D80.0
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary hypogammaglobulinemia
Short Description
Hereditary hypogammaglobulinemia
Same as the full description in the CMS dataset.
Parent Code
D80
Immunodeficiency with predominantly antibody defects

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD80-D89Certain disorders involving the immune mechanism
CategoryD80Immunodeficiency with predominantly antibody defects
This CodeD80.0Hereditary hypogammaglobulinemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome
  • Autosomal agammaglobulinemia with absent B-cells
  • Autosomal dominant agammaglobulinemia due to E47 transcription factor deficiency
  • Autosomal dominant agammaglobulinemia due to PU.1 deficiency
  • Autosomal recessive agammaglobulinemia
  • Autosomal recessive agammaglobulinemia due to BLNK deficiency
  • Autosomal recessive agammaglobulinemia due to E47 transcription factor deficiency
  • Autosomal recessive agammaglobulinemia due to FNIP1 deficiency
  • Autosomal recessive agammaglobulinemia due to immunoglobulin alpha deficiency
  • Autosomal recessive agammaglobulinemia due to immunoglobulin beta deficiency
  • Autosomal recessive agammaglobulinemia due to immunoglobulin heavy chain mu constant region deficiency
  • Autosomal recessive agammaglobulinemia due to lambda 5 deficiency
  • Autosomal recessive agammaglobulinemia due to p110 delta deficiency
  • Autosomal recessive agammaglobulinemia due to p85 deficiency
  • Autosomal recessive agammaglobulinemia due to SLC39A7 deficiency
  • B-cell immunodeficiency, limb anomaly, urogenital malformation syndrome
  • Congenital agammaglobulinemia
  • Congenital hypogammaglobulinemia
  • Hypogammaglobulinemia
  • Infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
  • Isolated agammaglobulinemia
  • Microcephaly, hypogammaglobulinemia, abnormal immunity syndrome
  • Osteopetrosis hypogammaglobulinemia syndrome
  • Proteinosis
  • Pulmonary alveolar proteinosis
  • Specific antibody deficiency
  • X-linked agammaglobulinemia
  • X-linked agammaglobulinemia with growth hormone deficiency
  • X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • 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

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD008
Immunity disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Pulmonary Alveolar Proteinosis

    a pulmonary alveoli-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and dyspnea. this disease is often related to, congenital or acquired, impaired processing of pulmonary surfactants by alveolar macrophages, a process dependent on granulocyte-macrophage colony-stimulating factor.

  • Pulmonary Alveolar Proteinosis

    a rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-schiff stain. it may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. signs and symptoms include dyspnea, cough and low grade fever.

  • Autoimmune Pulmonary Alveolar Proteinosis

    a type of pulmonary alveolar proteinosis associated with low concentrations of gm-csf.

Patient EducationClinical

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

The full article covers:

  • What is the immune system?
  • What are the parts of the immune system?
  • How does the immune system work?
  • What are the types of immunity?
  • What can go wrong with the immune system?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D80.0 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
279.04 Cong hypogammaglobulinem
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D80.0Overview

Is D80.0 (Immunodeficiency with predominantly antibody defects) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary hypogammaglobulinemia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D80.0 group to?

When hereditary hypogammaglobulinemia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 814, 815, 816, with relative weights from 0.6320 to 2.1267 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D80.0?

Under the General Equivalence Mappings, hereditary hypogammaglobulinemia converts to ICD-9-CM 279.04 (cong hypogammaglobulinem). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.