2026 ICD-10-CM Diagnosis Code D80.0Hereditary hypogammaglobulinemia
ICD-10-CM Codes›D50–D89›D80-D89›D80
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Risk Adjusts — HCC 115
- Chronic Condition
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. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 44 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.
For Medicare Advantage risk adjustment, D80.0 maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders) under the V28 model, adding a risk factor of about 0.565 for a community, non-dual, aged beneficiary in payment year 2026.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
D80.0 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2026.
Source: CMS Payment Year 2026 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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)
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Agammaglobulinemia (acquired (secondary)) (nonfamilial) D80.1
Bruton's X-linked D80.0
congenital sex-linked D80.0
hereditary D80.0
Hypogammaglobulinemia See Also: Agammaglobulinemia; D80.1
hereditary D80.0
Syndrome See Also: Disease;
hereditary D80.0
congenital D80.0
hereditary D80.0
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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.
Code HistoryHistory
Questions About D80.0Overview
What is the ICD-10 code for hereditary hypogammaglobulinemia?
The ICD-10-CM code for hereditary hypogammaglobulinemia is D80.0 (sometimes written as D800). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
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.
Is D80.0 a CC or MCC?
CMS lists D80.0 as a CC (complication or comorbidity) for FY 2026. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 44 closely related codes in its exclusion list.
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.
What HCC is D80.0?
D80.0 (hereditary hypogammaglobulinemia) maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders), commonly written as HCC 115, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2026. It mapped to HCC 47 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 99.
Does D80.0 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, D80.0 adds a risk adjustment factor of about 0.565 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.302 to 0.692 depending on the payment segment). A more severe related category (HCC 114) supersedes it when both are reported. See the full factor table on the HCC 115 category page.
