Skip to main content

D80.1 ICD-10-CM Code: Nonfamilial hypogammaglobulinemia

Compare with another codeCheck this code on a claim

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
6 inclusion terms · 2 Excludes1 · 1 Excludes2
Risk adjustment
CMS-HCC V22 category 47 · RxHCC V08 category 99

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v44, Appendix B.

  • MS-DRG 814 — RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH MCC (MDC 16)
  • MS-DRG 815 — RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH CC (MDC 16)
  • MS-DRG 816 — RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITHOUT CC/MCC (MDC 16)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D80.1 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 D80.1 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

Inclusion Terms

Alternative terms the tabular list files under this code.

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

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).

Source: inherited from D80-D89

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 D80.1

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

Before you code D80.1

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with D80.1. 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 a condition named in D80.1’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.

    ReviewM35.9, D71

Consider D80.1. Then confirm the code is valid for the date of service in the Verify section.

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).
The associated condition or complication
Whether the associated condition the title names is documented; the “with” convention presumes some links, and a provider statement that the conditions are unrelated defeats it (Guidelines I.A.15).

Official instructions as workflow

  • Excludes1 — check before selecting D80.1(2 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with D80.1: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareM35.9, D71

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

  • Excludes2 — not part of D80.1(1 note)

    Coding workflow: The conditions named in this note are not included in D80.1. 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 D80.1 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).

ReviewM35.9, 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.

Coding context

The comparisons, hierarchy, index entries, guidelines, relationships, risk-adjustment and coverage context a coder commonly needs. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Don’t confuse D80.1 with…

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

The codes most likely to be picked instead of D80.1 — 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.

  • 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: D80.1 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: D80.1 vs D71 →

  • D80.9— Specificity relationship (Review)

    Overlapping diagnoses in the same category.

    Overlapping codes in the same category — the documentation decides which is the more specific fit.

    What moves you there: A record that documents no more than “Immunodeficiency with predominantly antibody defects, unspecified” takes D80.9; specificity in the record moves it back here.

    Full comparison: D80.1 vs D80.9 →

  • 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: D80.1 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.

Code Overview

Nonfamilial hypogammaglobulinemia is a billable ICD-10-CM diagnosis code (D80.1).

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

Indexed Clinical Terms (11)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Official Coding Guidelines

No excerpt in the ICD-10-CM Official Guidelines names this code specifically. Its chapter carries only this chapter-wide note:

Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Reserved for future guideline expansion

Verify Before Coding

  • CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.

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 D80.1 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 2 Excludes1 notes: D72 — Other disorders of white blood cells (via D80.-), D84.81 — Immunodeficiency due to conditions classified elsewhere (via D80.-).

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

Referenced by 1 Code First instruction: M36.8 — Systemic disorders of connective tissue in other diseases classified elsewhere (via D80.-).

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 46 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 814 (MDC 16), DRG 815 (MDC 16), DRG 816 (MDC 16).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):BLD008 — Immunity disorders (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

D80.0 — Hereditary hypogammaglobulinemia, D80.2 — Selective deficiency of immunoglobulin A [IgA], D80.3 — Selective deficiency of immunoglobulin G [IgG] subclasses, D80.4 — Selective deficiency of immunoglobulin M [IgM], D80.5 — Immunodeficiency with increased immunoglobulin M [IgM], D80.6 — Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia, D80.7 — Transient hypogammaglobulinemia of infancy, D80.8 — Other immunodeficiencies with predominantly antibody defects, D80.9 — Immunodeficiency with predominantly antibody defects, unspecified, D81.0 — Severe combined immunodeficiency [SCID] with reticular dysgenesis, D81.1 — Severe combined immunodeficiency [SCID] with low T- and B-cell numbers, D81.2 — Severe combined immunodeficiency [SCID] with low or normal B-cell numbers, D81.4 — Nezelof's syndrome, D81.6 — Major histocompatibility complex class I deficiency, D81.7 — Major histocompatibility complex class II deficiency, D81.82 — Activated Phosphoinositide 3-kinase Delta Syndrome [APDS], D81.89 — Other combined immunodeficiencies, D81.9 — Combined immunodeficiency, unspecified, D82.0 — Wiskott-Aldrich syndrome, D82.1 — Di George's syndrome, +25 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Immunity disorders).

D80.0 — Hereditary hypogammaglobulinemia, D80.2 — Selective deficiency of immunoglobulin A [IgA], D80.3 — Selective deficiency of immunoglobulin G [IgG] subclasses, D80.4 — Selective deficiency of immunoglobulin M [IgM], D80.5 — Immunodeficiency with increased immunoglobulin M [IgM], D80.6 — Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia, D80.7 — Transient hypogammaglobulinemia of infancy, D80.8 — Other immunodeficiencies with predominantly antibody defects, D80.9 — Immunodeficiency with predominantly antibody defects, unspecified, D81.0 — Severe combined immunodeficiency [SCID] with reticular dysgenesis, D81.1 — Severe combined immunodeficiency [SCID] with low T- and B-cell numbers, D81.2 — Severe combined immunodeficiency [SCID] with low or normal B-cell numbers, D81.3 — Adenosine deaminase [ADA] deficiency, D81.30 — Adenosine deaminase deficiency, unspecified, D81.31 — Severe combined immunodeficiency due to adenosine deaminase deficiency, D81.32 — Adenosine deaminase 2 deficiency, D81.39 — Other adenosine deaminase deficiency, D81.4 — Nezelof's syndrome, D81.5 — Purine nucleoside phosphorylase [PNP] deficiency, D81.6 — Major histocompatibility complex class I deficiency, +64 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Deficiency, deficient”, “Agammaglobulinemia”, “Arthritis, arthritic”, …; these codes share that main term but sit in a different category of the Tabular List.

D68.2 — Hereditary deficiency of other clotting factors (fibrinogen), D68.4 — Acquired coagulation factor deficiency (prothrombin, acquired), D68.59 — Other primary thrombophilia (protein, S), D68.8 — Other specified coagulation defects (factor, multiple), D68.9 — Coagulation defect, unspecified (clotting), D69.0 — Allergic purpura (in, Henochpurpura), D69.19 — Other qualitative platelet defects (platelet NEC), D71.1 — Leukocyte adhesion deficiency (leukocyte adhesion), D74.0 — Congenital methemoglobinemia (NADH-methemoglobin reductase), D75.A — Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia (glucose-6-phosphate dehydrogenase, without anemia), D81.30 — Adenosine deaminase deficiency, unspecified (adenosine deaminase), D81.31 — Severe combined immunodeficiency due to adenosine deaminase deficiency (adenosine deaminase, with severe combined immunodeficiency), D81.32 — Adenosine deaminase 2 deficiency (ADA2), D81.39 — Other adenosine deaminase deficiency (adenosine deaminase, type 1), D81.5 — Purine nucleoside phosphorylase [PNP] deficiency (purine nucleoside phosphorylase), D81.6 — Major histocompatibility complex class I deficiency (major histocompatibility complex, class I), D81.7 — Major histocompatibility complex class II deficiency (major histocompatibility complex, class II), D81.810 — Biotinidase deficiency (biotinidase), D81.819 — Biotin-dependent carboxylase deficiency, unspecified (biotin-dependent carboxylase), D81.9 — Combined immunodeficiency, unspecified (lymphopenic), +576 more

Contextual Map

Every relationship of D80.1 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 D80.1 with these 3 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Referenced by Code First instructions

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 16 — Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders[MDC crossing]: “Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 1,615 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries (11)

  • Absence (of) (organ or part) (complete or partial), gamma globulin in blood[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Agammaglobulinemia (acquired (secondary)) (nonfamilial)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Agammaglobulinemia (acquired (secondary)) (nonfamilial), common variable (CVAgamma)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Agammaglobulinemia (acquired (secondary)) (nonfamilial), with, immunoglobulin-bearing B-lymphocytes[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute), in (due to), hypogammaglobulinemia[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Deficiency, deficient, gammaglobulin in blood[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disease, diseased, connective tissue, systemic (diffuse), in (due to), hypogammaglobulinemia[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Hypogammaglobulinemia[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • and 3 more

Nearest codes (10)

Change history (3)

Deep reference

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

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
Coding guidelines Official source data
ICD-10-CM Official Guidelines for Coding and Reporting (FY2027), quoted by section 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
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v44 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 20, 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. "D80.1 — Nonfamilial hypogammaglobulinemia." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/d80.1-nonfamilial-hypogammaglobulinemia

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
    Nonfamilial hypogammaglobulinemia

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D80.1 in its code family, with their registry titles.

View all codes in the D80 family