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D81 ICD-10-CM Code: Combined immunodeficiencies

Compare with another code for D81Check this code on a claim

Billing Status: NO. This is a non-billable ICD-10-CM code: report a more specific billable code beneath it.

Coding at a Glance

Official instructions
3 inclusion terms · 3 Excludes1 · 1 Excludes2

What these terms mean →

Category · FY2027A non-billable heading in the tabular list: report a more specific code beneath it.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too, and is marked with where it comes from.

Excludes1Not reported with this code unless documented as unrelated
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
  • autoimmune disease (systemic) NOS (M35.9) (from D80-D89)
  • functional disorders of polymorphonuclear neutrophils (D71-) (from D80-D89)
Excludes2from D80-D89Not included here; may be reported together
  • human immunodeficiency virus [HIV] disease (B20)
Includesfrom D80-D89What D80-D89 covers
  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Where it sits Source: CMS/NCHS

Under:Chapter 3: Diseases of the Blood and Blood-forming Organs and Certain Disorders Involving the Immune MechanismD80-D89 Certain disorders involving the immune mechanism

Codes under D81 (10)

  • D81.0Severe combined immunodeficiency [SCID] with reticular dysgenesisBillable
  • D81.1Severe combined immunodeficiency [SCID] with low T- and B-cell numbersBillable
  • D81.2Severe combined immunodeficiency [SCID] with low or normal B-cell numbersBillable
  • D81.3Adenosine deaminase [ADA] deficiencyNot billable
  • D81.4Nezelof's syndromeBillable
  • D81.5Purine nucleoside phosphorylase [PNP] deficiencyBillable
  • D81.6Major histocompatibility complex class I deficiencyBillable
  • D81.7Major histocompatibility complex class II deficiencyBillable
  • D81.8Other combined immunodeficienciesNot billable
  • D81.9Combined immunodeficiency, unspecifiedBillable

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D81 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on D81 itself; “inherited from” names the category or block whose note applies here.

Includes

Conditions the official ICD-10-CM tabular list includes under this code.

  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Source: inherited from D80-D89

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from D80-D89

Coder workflow for D81

MedCoder structured workflow — derived from this code’s own official record

Before you code D81

  1. D81 is not reportable as written. Select the more specific code beneath it that the documentation supports. Codes are reported to the highest level of specificity the classification provides (Guidelines I.B.2).

    ReviewD81.0, D81.1, D81.2, D81.3, D81.4, D81.5, D81.6, D81.7, D81.8, D81.9

    See the relationships section · Guide: How to choose an ICD-10-CM code →

  2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with the code beneath D81. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support one of the more specific codes beneath D81?
    Yes → Select that code and continue the checks below on its own page.
    No → D81 cannot be reported as written. Where the codes beneath it include an unspecified code, that code applies when the record goes no further; otherwise query for the missing detail.

    ReviewD81.0, D81.1, D81.2, D81.3, D81.4, D81.5, D81.6, D81.7, D81.8, D81.9

  2. Does the documentation support a condition named in D81’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewD80.0, M35.9, D71

Report the code beneath D81 that the documentation supports, not D81 itself. Then confirm that code is valid for the date of service on its own page.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).

Official instructions as workflow

  • Excludes1 — check before selecting D81(3 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with D81: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareD80.0, M35.9, D71

    See the official tabular notes · Guidelines I.A.12.a

  • Excludes2 — not part of D81(1 note)

    Coding workflow: The conditions named in this note are not included in D81. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareB20

    See the official tabular notes · Guidelines I.A.12.b

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition D81 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewD80.0, M35.9, D71

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Don’t confuse D81 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of D81 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • D80.0— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of autosomal recessive agammaglobulinemia (Swiss type) moves the case to D80.0; the two are not reported together unless documented as unrelated.

    Full comparison: D81 vs D80.0 →

  • M35.9— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of autoimmune disease (systemic) NOS moves the case to M35.9; the two are not reported together unless documented as unrelated.

    Full comparison: D81 vs M35.9 →

  • D71— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of functional disorders of polymorphonuclear neutrophils moves the case to D71; the two are not reported together unless documented as unrelated.

    Full comparison: D81 vs D71 →

  • B20— Excludes2 relationship (Informational)

    Distinct conditions — both may be reported.

    A distinct condition — both may be reported when both are documented.

    What moves you there: Documentation of human immunodeficiency virus [HIV] disease adds B20; it is not part of this code and both may be reported when both are documented.

    Full comparison: D81 vs B20 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Combined immunodeficiencies is a non-billable ICD-10-CM category code (D81). A more specific billable subcode must be selected for claims submission.

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name D81 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 3 Excludes1 notes across 2 chapters: D72 — Other disorders of white blood cells, D84.81 — Immunodeficiency due to conditions classified elsewhere, E79 — Disorders of purine and pyrimidine metabolism.

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Contextual Map

Every relationship of D81 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run D81 with these 7 related codes in Claim Check

Hierarchy

Excludes1

Excludes2

Referenced by Excludes1 notes

Nearest codes (20)

Change history (3)

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Common coding questions

MedCoder editorial

Can D81 be billed directly?

No. D81 (Combined immunodeficiencies) is a non-billable ICD-10-CM category code. A more specific billable subcode must be selected based on clinical documentation.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder-derived relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder editorial explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "D81 — Combined immunodeficiencies." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/d81-combined-immunodeficiencies

Change history

Changed in FY2027, the release in force: Excludes2 note added; Excludes1 note removed.

  • FY2027 — October 1, 2026
    Excludes2 note added
    human immunodeficiency virus [HIV] disease (B20)
    FY2027 changes
  • FY2027 — October 1, 2026
    Excludes1 note removed
    human immunodeficiency virus [HIV] disease (B20)
    FY2027 changes
  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Combined immunodeficiencies

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D81 in its code family, with their registry titles.