D81.89

Billable

Other combined immunodeficiencies

Other combined immunodeficiencies

Status

Billable / Specific

Block

D80-D89

Parent Code

D81.8

Coding Notes

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(2)
D81.81

Biotin-dependent carboxylase deficiency

D81.82

Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]

Billable
Also Known As / Clinical Terms(467)

SNOMED CT

UMLS

Clinical Terms

  • Progressive microcephaly, seizures, cortical blindness, developmental delay syndrome with combined immunodeficiency due to diaphanous related formin 1 mutation
  • Autosomal recessive combined immunodeficiency due to ITPKB mutation
  • Combined immunodeficiency due to RELA haploinsufficiency
  • Immunodeficiency by defective expression of major histocompatibility complex class I
  • Autosomal recessive combined immunodeficiency due to DNA polymerase delta 1 catalytic subunit mutation
  • Severe combined immunodeficiency due to DNA-PKcs deficiency
  • T-cell receptor alpha-beta-positive T-cell deficiency
  • Autosomal recessive deoxyribonucleic acid repair defect due to DNA ligase 1 deficiency
  • Primary immunodeficiency with multifaceted aberrant lymphoid immunity
  • Autosomal recessive combined immunodeficiency due to partial interleukin 6 cytokine family signal transducer deficiency
  • Autosomal recessive combined immunodeficiency due to RELB deficiency
  • TFRC (transferrin receptor) related combined immunodeficiency
  • Autosomal dominant combined immunodeficiency due to partial IL6ST deficiency
  • Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphoedema syndrome
  • Autosomal recessive combined immunodeficiency due to minichromosome maintenance complex component 10 deficiency
  • Hyper-IgE syndrome
  • Autosomal dominant hyperimmunoglobulin E syndrome due to ERBIN deficiency
  • Combined immunodeficiency due to Zeta-chain associated protein kinase 70 deficiency
  • Primary immunodeficiency with natural killer cell deficiency and adrenal insufficiency
  • T-cell negative B-cell positive severe combined immunodeficiency due to JAK3 deficiency
  • Autosomal recessive combined immunodeficiency due to RELB mutation
  • Autosomal recessive DNA repair defect due to polymerase-epsilon subunit 2 deficiency
  • Combined immunodeficiency due to caspase recruitment domain family member 11 protein deficiency
  • Severe combined immunodeficiency due to BCL11B deficiency
  • Severe combined immunodeficiency T-cell negative B-cell positive due to janus kinase-3 deficiency
  • Combined immunodeficiency due to CARMIL2 deficiency
  • Autosomal recessive combined immunodeficiency due to CD28 molecule mutation
  • Autosomal recessive deoxyribonucleic acid repair defect due to NSMCE3 deficiency
  • Combined immunodeficiency due to CD3-gamma deficiency
  • Lymphopenia
  • Autosomal recessive combined immunodeficiency due to inducible T-cell costimulator ligand deficiency
  • Autosomal recessive combined immunodeficiency due to POLD1 mutation
  • Autosomal dominant combined immunodeficiency due to IKZF3 mutation
  • Combined immunodeficiency due to moesin deficiency
  • Severe T-cell immunodeficiency, congenital alopecia, nail dystrophy syndrome
  • Autosomal dominant combined immunodeficiency due to partial interleukin 6 cytokine family signal transducer deficiency
  • Combined immunodeficiency due to transferrin receptor deficiency
  • Autosomal recessive combined immunodeficiency due to complete IL6 signal transducer protein deficiency
  • RNF168 (ring finger protein 168) deficiency
  • Autosomal recessive DNA repair defect due to LIG1 deficiency
  • Autosomal recessive DNA repair defect due to POLE2 deficiency
  • OL-EDA-ID (anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphoedema) syndrome
  • Hyperimmunoglobulin E syndrome
  • Severe combined immunodeficiency Athabascan type
  • Combined immunodeficiency due to FOXN1 haploinsufficiency
  • WHIM - Warts, hypogammaglobulinaemia, infections and myelokathexis
  • Developmental delay, immunodeficiency, leukoencephalopathy, hypohomocysteinemia syndrome
  • Immune dysfunction with T-cell inactivation due to calcium entry defect
  • Severe combined immunodeficiency due to deoxyribonucleic acid cross-link repair protein 1c deficiency
  • Combined immunodeficiency due to DOCK8 deficiency
  • Autosomal recessive combined immunodeficiency due to CHUK mutation
  • Autosomal recessive CID (combined immunodeficiency) due to ICOS deficiency
  • Autosomal recessive combined immunodeficiency due to paired box 1 mutation
  • CID-MIA/early-onset IBD - combined immunodeficiency-multiple intestinal atresia/early-onset inflammatory bowel disease
  • Combined immunodeficiency due to ITK deficiency
  • Warts, hypogammaglobulinaemia, infections, and myelokathexis
  • Autosomal recessive combined immunodeficiency due to Wiskott Aldrich syndrome protein-interacting protein deficiency
  • Combined immunodeficiency due to STK4 (serine/threonine kinase 4) deficiency
  • Autosomal recessive combined immunodeficiency due to ITPKB deficiency
  • Combined immunodeficiency due to dedicator of cytokinesis 8 deficiency
  • Combined immunodeficiency with childhood-onset Kaposi sarcoma
  • Combined immunodeficiency due to lipopolysaccharide-responsive beige-like anchor protein deficiency
  • WHIM - Warts, hypogammaglobulinemia, infections and myelokathexis
  • Combined immunodeficiency due to STK4 deficiency
  • Combined immunodeficiency with intrauterine growth retardation, NK (natural killer) cell deficiency, neutropenia
  • Autosomal dominant combined immunodeficiency due to STAT5b deficiency
  • Immunodeficiency by defective expression of human leucocyte antigen class I
  • Severe combined immunodeficiency due to IKK2 deficiency
  • Autosomal recessive combined immunodeficiency due to MAN2B2 mutation
  • Combined immunodeficiency due to interleukin-2 inducible T cell kinase deficiency
  • T-cell negative B-cell positive severe combined immunodeficiency
  • Autosomal dominant combined immunodeficiency due to STAT5b mutation
  • Combined immunodeficiency due to interleukin 21 receptor deficiency
  • Autosomal recessive DNA repair defect due to ligase 1 deficiency
  • Congenital alopecia and nail dystrophy with severe functional T-cell immunodeficiency
  • Stüve Wiedemann syndrome type 2
  • T-B+ (T-cell negative B-cell positive) severe combined immunodeficiency
  • Autosomal dominant combined immunodeficiency due to partial glycoprotein 130 deficiency
  • Autosomal recessive combined immunodeficiency due to BCL10 deficiency
  • Autosomal recessive combined immunodeficiency due to IL6R deficiency
  • Deficiency of DNA repair
  • Autosomal recessive combined immunodeficiency due to complete IL6ST deficiency
  • T-B+ SCID - T-cell negative B-cell positive severe combined immunodeficiency
  • Dedicator of cytokinesis 2 deficiency
  • MSN (moesin) related combined immunodeficiency
  • Combined immunodeficiency due to capping protein regulator and myosin 1 linker 2 deficiency
  • Severe combined immunodeficiency due to coronin 1A deficiency
  • BENTA (B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy) disease
  • Progressive microcephaly, seizures, cortical blindness, developmental delay syndrome
  • Developmental delay, immunodeficiency, leucoencephalopathy, hypohomocysteinemia syndrome
  • DOCK2 deficiency
  • B-cell expansion with NF-kB and T-cell anergy disease
  • Combined immunodeficiency due to CD3gamma deficiency
  • Combined immunodeficiency due to OX40 deficiency
  • Lymphocytopenia
  • Immunodeficiency by defective expression of HLA (human leukocyte antigen) class 1
  • Immunodeficiency by defective expression of HLA (human leucocyte antigen) class 1
  • RIDDLE syndrome
  • Severe combined immunodeficiency due to LAT (linker for activation of T cells) deficiency
  • Lymphocyte count below reference range
  • Combined immunodeficiency due to TTC7A mutation
  • Autosomal recessive combined immunodeficiency with multiple intestinal atresias
  • BENTA disease
  • Autosomal recessive hyperimmunoglobulin E syndrome due to partial IL6 signal transducer deficiency
  • Autosomal recessive combined immunodeficiency due to GP130 deficiency
  • Primary immunodeficiency due to MCM4 (mini-chromosome maintenance complex component 4) deficiency
  • Autosomal recessive combined immunodeficiency due to DNA polymerase delta 2 accessory subunit mutation
  • Familial CD8 deficiency
  • Autosomal recessive hyperimmunoglobulin E syndrome due to complete IL6ST deficiency
  • Combined immunodeficiency due to FCHO1 deficiency
  • Autosomal dominant combined immunodeficiency due to Aiolos deficiency
  • Autosomal recessive combined immunodeficiency due to IL6 receptor protein deficiency
  • Combined immunodeficiency due to calcium release activated calcium channel dysfunction
  • Severe combined immunodeficiency due to LAT deficiency
  • Warts, hypogammaglobulinemia, infections, and myelokathexis
  • Autosomal recessive combined immunodeficiency due to complete glycoprotein 130 deficiency
  • Combined immunodeficiency due to IL21R deficiency
  • Combined immunodeficiency due to forkhead box N1 haploinsufficiency
  • Combined immunodeficiency due to dedicator of cytokinesis 8 protein deficiency
  • Combined immunodeficiency due to RLTPR deficiency
  • Autosomal recessive combined immunodeficiency due to WIPF1 mutation
  • Autosomal recessive combined immunodeficiency due to ICOS deficiency
  • Autosomal recessive combined immunodeficiency due to COPG1 deficiency
  • Amino acid below reference range
  • Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema syndrome
  • Bare lymphocyte syndrome type 1
  • Autosomal recessive combined immunodeficiency due to POLD2 mutation
  • TCR (T-cell receptor) alpha-beta-positive T-cell deficiency
  • Combined immunodeficiency due to IL2 inducible T cell kinase deficiency
  • Autosomal recessive combined immunodeficiency due to complete interleukin 6 cytokine family signal transducer deficiency
  • Autosomal recessive lymphoproliferative disease due to ITK deficiency
  • Autosomal dominant combined immunodeficiency due to partial interleukin 6 signal transducer protein deficiency
  • Combined immunodeficiency due to GINS1 deficiency
  • Immunodeficiency by defective expression of human leukocyte antigen class I
  • Progressive microcephaly, seizures, cortical blindness, developmental delay with combined immunodeficiency due to DIAPH1 mutation
  • MHC (major histocompatibility complex) class I deficiency
  • Autosomal recessive combined immunodeficiency due to c-Rel deficiency
  • Major histocompatibility complex class I deficiency
  • Severe combined immunodeficiency due to artemis deficiency
  • Immunodeficiency by defective expression of human leukocyte antigen class 1
  • Combined immunodeficiency due to GINS complex subunit 1 deficiency
  • Alymphoid cystic thymic dysgenesis
  • Alymphocytosis
  • Autosomal dominant combined immunodeficiency due to erbb2 interacting protein deficiency
  • SCBMS (seizures, cortical blindness, microcephaly syndrome) with combined immunodeficiency due to DIAPH1 mutation
  • Autosomal recessive combined immunodeficiency due to COPI coat complex subunit gamma 1 deficiency
  • Combined immunodeficiency due to ZAP70 (Zeta-chain associated protein kinase 70) deficiency
  • Combined immunodeficiency due to ZAP70 deficiency
  • OL-EDA-ID (anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema) syndrome
  • Autosomal recessive hyperimmunoglobulin E syndrome due to IL6R deficiency
  • DOCK8 immunodeficiency syndrome
  • Autosomal recessive combined immunodeficiency due to mannosidase alpha class 2B member 2 mutation
  • Winged helix deficiency
  • X-linked combined immunodeficiency due to SAM and SH3 domain containing 3 deficiency
  • Autosomal recessive deoxyribonucleic acid repair defect due to NSMCE3 mutation
  • Combined immunodeficiency due to CD70 deficiency
  • Autosomal recessive combined immunodeficiency due to IKK-alpha deficiency
  • Autosomal dominant hyperimmunoglobulin E syndrome due to ERBB2IP defect
  • HIES - hyperimmunoglobulin E syndrome
  • Progressive microcephaly, seizures, cortical blindness, developmental delay with combined immunodeficiency due to DIAPH1 deficiency
  • RIDDLE (radiosensitivity, immunodeficiency, dysmorphic features, learning difficulties) syndrome
  • Combined immunodeficiency due to IKAROS family zinc finger 2 mutation
  • Autosomal recessive combined immunodeficiency due to PAX1 deficiency
  • Autosomal recessive combined immunodeficiency due to CD28 mutation
  • Pignata Guarino syndrome
  • Susceptibility to respiratory infection associated with CD8alpha chain mutation
  • Severe combined immunodeficiency due to CORO1A deficiency
  • Combined immunodeficiency due to MALT1 deficiency
  • Combined immunodeficiency due to HELIOS deficiency
  • Autosomal dominant combined immunodeficiency due to signal transducer and activator of transcription 5B mutation
  • Autosomal recessive combined immunodeficiency due to ICOSLG deficiency
  • Primary immunodeficiency due to MCM4 deficiency
  • Severe combined immunodeficiency due to inhibitor of nuclear factor kappa B kinase subunit beta deficiency
  • Combined immunodeficiency due to LRBA deficiency
  • Decreased amino acid
  • DNA repair
  • T-cell immunodeficiency, congenital alopecia, and nail dystrophy
  • Autosomal recessive combined immunodeficiency due to BCL10 mutation
  • Autosomal recessive deoxyribonucleic acid repair defect due to DNA polymerase epsilon 2, accessory subunit deficiency
  • Immunodeficiency by defective expression of human leucocyte antigen class 1
  • Severe combined immunodeficiency due to BCL11 transcription factor B deficiency
  • Autosomal recessive combined immunodeficiency due to MAN2B2 deficiency
  • Combined immunodeficiency due to CARD11 deficiency
  • Severe combined immunodeficiency due to deoxyribonucleic acid dependent protein kinase catalytic subunit deficiency
  • Combined immunodeficiency due to CRAC channel dysfunction
  • Combined immunodeficiency due to IKZF2 mutation
  • NF-kappa-B-inducing kinase deficiency
  • X-linked combined immunodeficiency due to SASH3 deficiency
  • Combined immunodeficiency due to mucosa-associated lymphoid tissue lymphoma translocation gene 1 deficiency
  • Autosomal recessive combined immunodeficiency due to REL proto-oncogene NF-kappa-B subunit mutation
  • Autosomal recessive combined immunodeficiency due to interleukin 6 receptor deficiency
  • Autosomal recessive combined immunodeficiency due to partial IL6 signal transducer protein deficiency
  • Combined immunodeficiency due to serine/threonine kinase 4 deficiency
  • NIK deficiency
  • Bare lymphocyte syndrome type I
  • Autosomal recessive combined immunodeficiency due to partial GP130 deficiency
  • X-linked moesin-associated immunodeficiency
  • Severe combined immunodeficiency due to linker for activation of T cells deficiency
  • Combined immunodeficiency due to CRAC (calcium release activated calcium) channel dysfunction
  • Autosomal recessive combined immunodeficiency due to partial glycoprotein 130 deficiency
  • Autosomal recessive combined immunodeficiency due to PAX1 mutation
  • B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy disease
  • Deoxyribonucleic acid repair
  • Radiosensitivity, immunodeficiency, dysmorphic features, learning difficulties syndrome
  • Autosomal recessive combined immunodeficiency due to Arp2/3-mediated filament branching defect
  • Bent bone dysplasia group
  • Autosomal dominant combined immunodeficiency due to ERBIN deficiency
  • Autosomal recessive combined immunodeficiency due to WASP-interacting protein deficiency
  • Combined immunodeficiency, enteropathy spectrum
  • LICS syndrome
  • Autosomal dominant hyperimmunoglobulin E syndrome due to partial IL6ST deficiency
  • Autosomal recessive combined immunodeficiency due to REL mutation
  • Myelokathexis
  • Autosomal recessive combined immunodeficiency due to MCM10 deficiency
  • Cortical blindness
  • Autosomal dominant combined immunodeficiency due to partial GP130 deficiency
  • Autosomal recessive combined immunodeficiency due to RELB proto-oncogene, NF-kB subunit mutation
  • Combined immunodeficiency due to TFRC deficiency
  • Autosomal recessive combined immunodeficiency due to component of inhibitor of nuclear factor kappa B kinase complex mutation
  • Autosomal recessive combined immunodeficiency due to BCL10 immune signaling adaptor mutation
  • Autosomal recessive combined immunodeficiency due to inositol-trisphosphate 3-kinase B mutation
  • Autosomal recessive combined immunodeficiency due to inducible T cell costimulator deficiency
  • Autosomal recessive combined immunodeficiency due to partial IL6ST deficiency
  • Autosomal recessive combined immunodeficiency due to WIP deficiency
  • Autosomal recessive DNA repair defect due to MCM10 deficiency
  • Autosomal recessive combined immunodeficiency due to ARPC1B mutation
  • Lung disease, immunodeficiency, chromosome breakage syndrome
  • Combined immunodeficiency due to FCH and mu domain containing endocytic adaptor 1 deficiency
  • Combined immunodeficiency due to GINS1 (GINS complex subunit 1) deficiency
  • DNA repair defect due to NSMCE3 deficiency
  • Deficiency of deoxyribonucleic acid repair
  • Severe combined immunodeficiency due to DCLRE1C deficiency
Frequently Asked Questions
What is the ICD-10 code for other combined immunodeficiencies?

The ICD-10-CM code for other combined immunodeficiencies is D81.89. The full clinical description is "Other combined immunodeficiencies". D81.89 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code D81.89 mean?

ICD-10-CM code D81.89 represents “Other combined immunodeficiencies”. 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 D81.89 a billable code?

Yes, D81.89 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is D81.89 in?

D81.89 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).

What codes cannot be used with D81.89?

D81.89 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); and 1 more.

What SNOMED CT codes does D81.89 map to?

D81.89 maps to 79 SNOMED CT concepts: 48813009, 720345008, 124057005, 720986005, 1350218008, and 74 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for D81.89?

D81.89 is linked to 1 UMLS Concept Unique Identifier: C0477324. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does D81.89 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other combined immunodeficiencies 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 D81.89?

There is no direct ICD-11 mapping available for D81.89 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

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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.