AutoICD API

D80.0

Billable

Hereditary hypogammaglobulinemia

Hereditary hypogammaglobulinemia

Status

Billable / Specific

Block

D80-D89

Parent Code

D80

ICD-11 Mapping

1 equivalent

Coding Notes

Inclusion Terms

Alternative clinical terms for this condition

  • Autosomal recessive agammaglobulinemia (Swiss type)
  • X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)

Includes

Conditions included under this code

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

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(9)
D80.1

Nonfamilial hypogammaglobulinemia

BillableD80.2

Selective deficiency of immunoglobulin A [IgA]

BillableD80.3

Selective deficiency of immunoglobulin G [IgG] subclasses

BillableD80.4

Selective deficiency of immunoglobulin M [IgM]

BillableD80.5

Immunodeficiency with increased immunoglobulin M [IgM]

BillableD80.6

Antibody defic w near-norm immunoglob or w hyperimmunoglob

BillableD80.7

Transient hypogammaglobulinemia of infancy

BillableD80.8

Other immunodeficiencies with predominantly antibody defects

BillableD80.9

Immunodeficiency with predominantly antibody defects, unsp

Billable
ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

4A01.00Hereditary agammaglobulinaemia with profoundly reduced or absent B cellsequivalentPrimary
Also Known As / Clinical Terms(235)

SNOMED CT

UMLS

Clinical Terms

  • Autosomal recessive agammaglobulinaemia due to FNIP1 deficiency
  • Autosomal recessive agammaglobulinemia due to immunoglobulin heavy chain mu constant region deficiency
  • Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome
  • Agammaglobulinemia due to SPI1 defect
  • Autosomal recessive agammaglobulinemia due to IGHM mutation
  • Autosomal agammaglobulinaemia with absent B-cells
  • Autosomal recessive agammaglobulinaemia due to PIK3R1 mutation
  • Agammaglobulinaemia due to SPI1 defect
  • Autosomal recessive agammaglobulinemia due to p110 delta deficiency
  • Bruton's hypogammaglobulinemia
  • Autosomal recessive agammaglobulinaemia due to solute carrier family 39 member 7 deficiency
  • Autosomal recessive agammaglobulinemia due to SLC39A7 deficiency
  • Proteinosis
  • Autosomal recessive osteopetrosis type 7
  • BILU (B-cell immunodeficiency, limb, urogenital) syndrome
  • OAS1-related infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinaemia
  • Functional antibody defect
  • Autosomal recessive agammaglobulinaemia
  • X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome
  • Osteopetrosis hypogammaglobulinaemia syndrome
  • Agammaglobulinaemia due to FNIP1 defect
  • PAP - Pulmonary alveolar proteinosis
  • BTK-deficiency
  • Agammaglobulinaemia, microcephaly, craniosynostosis, severe dermatitis syndrome
  • Isolated agammaglobulinaemia
  • Microcephaly, hypogammaglobulinemia, abnormal immunity syndrome
  • Autosomal recessive agammaglobulinemia due to B cell linker deficiency
  • Autosomal recessive agammaglobulinaemia due to E47 transcription factor deficiency
  • X-linked intellectual disability with hypogammaglobulinaemia and progressive neurological deterioration syndrome
  • Isolated agammaglobulinemia
  • Autosomal recessive agammaglobulinemia
  • Hoffman syndrome due to TOP2B deficiency
  • Agammaglobulinaemia due to TOP2B mutation
  • Autosomal recessive agammaglobulinemia due to immunoglobulin beta deficiency
  • Bruton's type agammaglobulinaemia
  • Specific antibody deficiency
  • X-linked agammaglobulinaemia with growth hormone deficiency
  • Autosomal recessive agammaglobulinemia due to IGLL1 mutation
  • Bruton's agammaglobulinaemia
  • Autosomal recessive agammaglobulinaemia due to p85 deficiency
  • Autosomal dominant agammaglobulinaemia due to TCF3 mutation
  • Osteopetrosis hypogammaglobulinemia syndrome
  • Autosomal recessive agammaglobulinaemia due to SLC39A7 deficiency
  • X-linked agammaglobulinaemia
  • Bruton tyrosine kinase deficiency
  • Autosomal recessive agammaglobulinaemia due to IGLL1 mutation
  • Congenital agammaglobulinemia
  • Autosomal recessive agammaglobulinaemia due to BLNK deficiency
  • B-cell immunodeficiency, limb anomaly, urogenital malformation syndrome
  • Pulmonary alveolar lipoproteinosis
  • Hypogammaglobulinemia
  • Autosomal recessive osteoclast-poor osteopetrosis with hypogammaglobulinaemia
  • Microcephaly, hypogammaglobulinaemia, abnormal immunity syndrome
  • Infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
  • Autosomal recessive agammaglobulinaemia due to B cell linker deficiency
  • Autosomal recessive agammaglobulinemia due to PIK3CD mutation
  • Fleisher syndrome
  • X-linked agammaglobulinemia
  • Autosomal recessive agammaglobulinaemia due to PIK3CD mutation
  • Bruton's hypogammaglobulinaemia
  • Autosomal recessive osteoclast-poor osteopetrosis with hypogammaglobulinemia
  • XLA - X-linked agammaglobulinaemia
  • Autosomal recessive agammaglobulinaemia due to immunoglobulin alpha deficiency
  • Alveolar proteinosis
  • Autosomal recessive agammaglobulinemia due to p85 deficiency
  • Autosomal dominant agammaglobulinemia due to TCF3 mutation
  • Autosomal recessive agammaglobulinemia due to PIK3R1 mutation
  • 2'-5'-oligoadenylate synthetase 1-related infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinaemia
  • Autosomal recessive agammaglobulinaemia due to folliculin interacting protein 1 deficiency
  • Autosomal recessive agammaglobulinaemia due to immunoglobulin heavy chain mu constant region deficiency
  • Bruton's type agammaglobulinemia
  • Autosomal recessive agammaglobulinemia due to immunoglobulin alpha deficiency
  • Agammaglobulinemia due to TOP2B mutation
  • 2'-5'-oligoadenylate synthetase 1-related infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
  • Congenital hypogammaglobulinemia
  • Infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinaemia
  • Autosomal dominant agammaglobulinemia due to E47 transcription factor deficiency
  • Autosomal recessive agammaglobulinaemia due to lambda 5 deficiency
  • Autosomal recessive agammaglobulinaemia due to IGHM mutation
  • Autosomal recessive agammaglobulinemia due to FNIP1 deficiency
  • Autosomal recessive agammaglobulinaemia due to BLNK mutation
  • BILU syndrome
  • Say Barber Miller syndrome
  • Specific immunoglobulin response defect
  • Autosomal recessive agammaglobulinemia due to CD79A mutation
  • Autosomal recessive agammaglobulinemia (Swiss type)
  • Congenital agammaglobulinaemia
  • X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)
  • Autosomal recessive agammaglobulinemia due to E47 transcription factor deficiency
  • Autosomal dominant agammaglobulinaemia due to PU.1 deficiency
  • Autosomal recessive agammaglobulinemia due to BLNK mutation
  • Autosomal recessive agammaglobulinemia due to BLNK deficiency
  • Autosomal recessive agammaglobulinaemia due to CD79B mutation
  • Autosomal dominant agammaglobulinaemia due to E47 transcription factor deficiency
  • Agammaglobulinemia due to FNIP1 defect
  • Pulmonary alveolar proteinosis
  • Autosomal recessive agammaglobulinaemia due to p110 delta deficiency
  • OAS1-related infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
  • Autosomal recessive agammaglobulinemia due to solute carrier family 39 member 7 deficiency
  • Autosomal recessive agammaglobulinemia due to folliculin interacting protein 1 deficiency
  • X-linked agammaglobulinemia with growth hormone deficiency
  • Bruton's agammaglobulinemia
  • X linked agammaglobulinemia
  • Autosomal recessive agammaglobulinemia due to TCF3 mutation
  • Short stature due to isolated growth hormone deficiency with X-linked hypogammaglobulinaemia
  • Autosomal agammaglobulinemia with absent B-cells
  • Autosomal dominant agammaglobulinemia due to PU.1 deficiency
  • Autosomal recessive agammaglobulinemia due to CD79B mutation
  • X linked agammaglobulinaemia
  • Autosomal recessive agammaglobulinaemia due to immunoglobulin beta deficiency
  • Congenital hypogammaglobulinaemia
  • Short stature due to isolated growth hormone deficiency with X-linked hypogammaglobulinemia
  • XLA - X-linked agammaglobulinemia
  • Autosomal recessive agammaglobulinaemia due to CD79A mutation
  • Autosomal recessive agammaglobulinaemia due to TCF3 mutation
  • Autosomal recessive agammaglobulinemia due to lambda 5 deficiency
  • Hypogammaglobulinaemia
Frequently Asked Questions
What is the ICD-10 code for hereditary hypogammaglobulinemia?

The ICD-10-CM code for hereditary hypogammaglobulinemia is D80.0. The full clinical description is "Hereditary hypogammaglobulinemia". D80.0 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code D80.0 mean?

ICD-10-CM code D80.0 represents “Hereditary hypogammaglobulinemia”. It is classified under Chapter 3: Diseases of the Blood and Blood-Forming Organs and is a billable/specific code that can be used on a claim.

Is D80.0 a billable code?

Yes, D80.0 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is D80.0 in?

D80.0 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).

What codes cannot be used with D80.0?

D80.0 has Excludes1 notes indicating codes that cannot be used together with it, including: autoimmune disease (systemic) NOS (M35.9); functional disorders of polymorphonuclear neutrophils (D71); human immunodeficiency virus [HIV] disease (B20).

What SNOMED CT codes does D80.0 map to?

D80.0 maps to 29 SNOMED CT concepts: 1197476009, 1351648007, 1351647002, 1230295000, 722281001, and 24 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for D80.0?

D80.0 is linked to 3 UMLS Concept Unique Identifiers: C2873841, C0494249, C2873842. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does D80.0 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like hereditary hypogammaglobulinemia affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of D80.0?

D80.0 maps to the ICD-11 code: 4A01.00 (Hereditary agammaglobulinaemia with profoundly reduced or absent B cells).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.