2026 ICD-10-CM Diagnosis Code D80.1Nonfamilial hypogammaglobulinemia

ICD-10-CM Codes›D50–D89›D80-D89›D80

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

D80.1 is a billable ICD-10-CM diagnosis code for nonfamilial 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. Coders also document this condition as agammaglobulinemia. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

Code Identity

ICD-10-CM Code
D80.1
Billable Status
Yes — Valid for Submission
Code Describes
Nonfamilial hypogammaglobulinemia
Short Description
Nonfamilial 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 CodeD80.1Nonfamilial hypogammaglobulinemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Agammaglobulinemia
  • B-lymphocyte immunodeficiency
  • Chromosome 22 abnormalities with hypogammaglobulinemia
  • Deletion of X-chromosome and hypogammaglobulinemia
  • Hypogammaglobulinemia
  • Hypogammaglobulinemia due to monoclonal gammopathy of undetermined significance
  • Hypogammaglobulinemia due to multiple myeloma
  • Triple X syndrome, epilepsy, and hypogammaglobulinemia

Tabular List NotesGuidance

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

Inclusion Terms

  • Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes
  • Common variable agammaglobulinemia CVAgamma
  • Hypogammaglobulinemia NOS

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
    • Agammaglobulinemia(acquired (secondary)) (nonfamilial)
    • Agammaglobulinemia(acquired (secondary)) (nonfamilial)
      • with
        • immunoglobulin-bearing B-lymphocytes
    • Agammaglobulinemia(acquired (secondary)) (nonfamilial)
      • common variable (CVAgamma)
    • Arthritis, arthritic(acute) (chronic) (nonpyogenic) (subacute)
      • in (due to)
        • hypogammaglobulinemia
    • Deficiency, deficient
      • gammaglobulin in blood
    • Disease, diseased
      • connective tissue, systemic (diffuse)
        • in (due to)
          • hypogammaglobulinemia
    • Hypogammaglobulinemia
    • Hypogammaglobulinemia
      • nonfamilial
    • Syndrome
      • antibody deficiency
        • agammaglobulinemic
    • Syndrome
      • antibody deficiency
        • hypogammaglobulinemic

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

  • Agammaglobulinemia

    an immunologic deficiency state characterized by an extremely low level of generally all classes of gamma-globulin in the blood.

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 D80.1 to ICD-9-CMHistory

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

ICD-9-CM
279.00 Hypogammaglobulinem NOS
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 D80.1Overview

Is D80.1 (Immunodeficiency with predominantly antibody defects) a billable code?

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

What MS-DRG does D80.1 group to?

When nonfamilial 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 D80.1?

Under the General Equivalence Mappings, nonfamilial hypogammaglobulinemia converts to ICD-9-CM 279.00 (hypogammaglobulinem NOS). 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.