2026 ICD-10-CM Diagnosis Code D81.89Other combined immunodeficiencies

ICD-10-CM Codes›D50–D89›D80-D89›D81

ICD-10-CM D81.89
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D81.89 is a billable ICD-10-CM diagnosis code for other combined immunodeficiencies. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 808 through 810. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

Code Identity

ICD-10-CM Code
D81.89
Billable Status
Yes — Valid for Submission
Code Describes
Other combined immunodeficiencies
Short Description
Other combined immunodeficiencies
Same as the full description in the CMS dataset.
Parent Code
D81.8
Other combined immunodeficiencies

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
CategoryD81Combined immunodeficiencies
This CodeD81.89Other combined immunodeficiencies

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Amino acid below reference range
  • Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema syndrome
  • Autosomal dominant combined immunodeficiency due to Aiolos deficiency
  • Autosomal dominant combined immunodeficiency due to ERBIN deficiency
  • Autosomal dominant combined immunodeficiency due to partial IL6ST deficiency
  • Autosomal dominant combined immunodeficiency due to STAT5b mutation
  • Autosomal recessive combined immunodeficiency due to Arp2/3-mediated filament branching defect
  • Autosomal recessive combined immunodeficiency due to BCL10 mutation
  • Autosomal recessive combined immunodeficiency due to CD28 mutation
  • Autosomal recessive combined immunodeficiency due to CHUK mutation
  • Autosomal recessive combined immunodeficiency due to complete IL6ST deficiency
  • Autosomal recessive combined immunodeficiency due to COPG1 deficiency
  • Autosomal recessive combined immunodeficiency due to ICOS deficiency
  • Autosomal recessive combined immunodeficiency due to ICOSLG deficiency
  • Autosomal recessive combined immunodeficiency due to IL6R deficiency
  • Autosomal recessive combined immunodeficiency due to ITPKB mutation
  • Autosomal recessive combined immunodeficiency due to MAN2B2 mutation
  • Autosomal recessive combined immunodeficiency due to MCM10 deficiency
  • Autosomal recessive combined immunodeficiency due to partial IL6ST deficiency
  • Autosomal recessive combined immunodeficiency due to PAX1 mutation
  • Autosomal recessive combined immunodeficiency due to POLD1 mutation
  • Autosomal recessive combined immunodeficiency due to POLD2 mutation
  • Autosomal recessive combined immunodeficiency due to REL mutation
  • Autosomal recessive combined immunodeficiency due to RELB mutation
  • Autosomal recessive combined immunodeficiency due to WIP deficiency
  • Autosomal recessive DNA repair defect due to LIG1 deficiency
  • Autosomal recessive DNA repair defect due to POLE2 deficiency
  • Bent bone dysplasia group
  • BENTA disease
  • Combined immunodeficiency due to CARD11 deficiency
  • Combined immunodeficiency due to CARMIL2 deficiency
  • Combined immunodeficiency due to CD3gamma deficiency
  • Combined immunodeficiency due to CD70 deficiency
  • Combined immunodeficiency due to CRAC channel dysfunction
  • Combined immunodeficiency due to DOCK8 deficiency
  • Combined immunodeficiency due to FCHO1 deficiency
  • Combined immunodeficiency due to FOXN1 haploinsufficiency
  • Combined immunodeficiency due to GINS1 deficiency
  • Combined immunodeficiency due to IKZF2 mutation
  • Combined immunodeficiency due to interleukin 21 receptor deficiency
  • Combined immunodeficiency due to ITK deficiency
  • Combined immunodeficiency due to LRBA deficiency
  • Combined immunodeficiency due to MALT1 deficiency
  • Combined immunodeficiency due to moesin deficiency
  • Combined immunodeficiency due to OX40 deficiency
  • Combined immunodeficiency due to RELA haploinsufficiency
  • Combined immunodeficiency due to STK4 deficiency
  • Combined immunodeficiency due to TFRC deficiency
  • Combined immunodeficiency due to ZAP70 deficiency
  • Combined immunodeficiency, enteropathy spectrum
  • Cortical blindness
  • Deficiency of DNA repair
  • Developmental delay, immunodeficiency, leukoencephalopathy, hypohomocysteinemia syndrome
  • DNA repair
  • DOCK2 deficiency
  • Hyperimmunoglobulin E syndrome
  • Lung disease, immunodeficiency, chromosome breakage syndrome
  • Lymphocyte count below reference range
  • Lymphocytopenia
  • Major histocompatibility complex class I deficiency
  • Myelokathexis
  • Primary immunodeficiency with multifaceted aberrant lymphoid immunity
  • Primary immunodeficiency with natural killer cell deficiency and adrenal insufficiency
  • Progressive microcephaly, seizures, cortical blindness, developmental delay syndrome
  • Progressive microcephaly, seizures, cortical blindness, developmental delay with combined immunodeficiency due to DIAPH1 mutation
  • RIDDLE syndrome
  • Severe combined immunodeficiency due to BCL11B deficiency
  • Severe combined immunodeficiency due to CORO1A deficiency
  • Severe combined immunodeficiency due to DCLRE1C deficiency
  • Severe combined immunodeficiency due to deoxyribonucleic acid dependent protein kinase catalytic subunit deficiency
  • Severe combined immunodeficiency due to IKK2 deficiency
  • Severe combined immunodeficiency due to LAT deficiency
  • Severe T-cell immunodeficiency, congenital alopecia, nail dystrophy syndrome
  • Susceptibility to respiratory infection associated with CD8alpha chain mutation
  • T-cell negative B-cell positive severe combined immunodeficiency
  • T-cell negative B-cell positive severe combined immunodeficiency due to JAK3 deficiency
  • T-cell receptor alpha-beta-positive T-cell deficiency
  • Warts, hypogammaglobulinemia, infections, and myelokathexis
  • X-linked combined immunodeficiency due to SASH3 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.

    • Immunodeficiency
      • combined
        • specified type NEC

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

  • DNA End-Joining Repair

    the repair of double-strand dna breaks by rejoining the broken ends of dna to each other directly.

  • DNA Repair

    the removal of dna lesions and/or restoration of intact dna strands without base pair mismatches, intrastrand or interstrand crosslinks, or discontinuities in the dna sugar-phosphate backbones.

  • DNA Repair Enzymes

    enzymes that are involved in the reconstruction of a continuous two-stranded dna molecule without mismatch from a molecule, which contained damaged regions.

  • DNA Repair-Deficiency Disorders

    disorders resulting from defective dna repair processes or the associated cellular responses to dna damage.

  • O(6)-Methylguanine-DNA Methyltransferase

    an enzyme that transfers methyl groups from o(6)-methylguanine, and other methylated moieties of dna, to a cysteine residue in itself, thus repairing alkylated dna in a single-step reaction. ec 2.1.1.63.

  • Rad52 DNA Repair and Recombination Protein

    a dna-binding protein that mediates dna repair of double strand breaks, and homologous recombination.

  • Recombinational DNA Repair

    repair of dna damage by exchange of dna between matching sequences, usually between the allelic dna (alleles) of sister chromatids.

  • DNA

    a deoxyribonucleotide polymer that is the primary genetic material of all cells. eukaryotic and prokaryotic organisms normally contain dna in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. dna, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).

  • Cortical Blindness

    visual impairment due to visual cortex dysfunction.

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 D81.89 to ICD-9-CMHistory

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

ICD-9-CM
279.2 Combined immunity defic
Approximate The match is approximate rather than exact.

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 D81.89Overview

Is D81.89 (Other combined immunodeficiencies) a billable code?

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

What MS-DRG does D81.89 group to?

When other combined immunodeficiencies is the principal diagnosis on an inpatient stay, it groups to MS-DRG 808, 809, 810, with relative weights from 1.0466 to 2.2079 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D81.89?

Under the General Equivalence Mappings, other combined immunodeficiencies converts to ICD-9-CM 279.2 (combined immunity defic). The mapping is approximate, so confirm the match fits the documentation.

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.