D81.89
BillableOther combined immunodeficiencies
Other combined immunodeficiencies
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
- autoimmune disease (systemic) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Also Known As / Clinical Terms
SNOMED CT
- Myelokathexis24974008
- Deficiency of DNA repair28965003
- Deficiency of deoxyribonucleic acid repair28965003
- Alymphocytosis48813009
- Lymphocytopenia48813009
- Lymphopenia48813009
- DNA repair65113001
- Deoxyribonucleic acid repair65113001
- Cortical blindness68574006
- Amino acid below reference range124057005
- Decreased amino acid124057005
- HIES - hyperimmunoglobulin E syndrome191009009
- Hyper-IgE syndrome191009009
- Hyperimmunoglobulin E syndrome191009009
- WHIM - Warts, hypogammaglobulinaemia, infections and myelokathexis234571003
- WHIM - Warts, hypogammaglobulinemia, infections and myelokathexis234571003
- Warts, hypogammaglobulinaemia, infections, and myelokathexis234571003
- Warts, hypogammaglobulinemia, infections, and myelokathexis234571003
- Bent bone dysplasia group278832007
- Severe combined immunodeficiency Athabascan type715982006
- Severe combined immunodeficiency due to DCLRE1C deficiency715982006
- Severe combined immunodeficiency due to artemis deficiency715982006
- Severe combined immunodeficiency due to deoxyribonucleic acid cross-link repair protein 1c deficiency715982006
- Combined immunodeficiency due to ZAP70 (Zeta-chain associated protein kinase 70) deficiency716378008
- Combined immunodeficiency due to ZAP70 deficiency716378008
- Combined immunodeficiency due to Zeta-chain associated protein kinase 70 deficiency716378008
- Severe combined immunodeficiency due to DNA-PKcs deficiency716871006
- Severe combined immunodeficiency due to deoxyribonucleic acid dependent protein kinase catalytic subunit deficiency716871006
- Combined immunodeficiency due to CRAC (calcium release activated calcium) channel dysfunction717811007
- Combined immunodeficiency due to CRAC channel dysfunction717811007
- Combined immunodeficiency due to calcium release activated calcium channel dysfunction717811007
- Immune dysfunction with T-cell inactivation due to calcium entry defect717811007
- Severe combined immunodeficiency T-cell negative B-cell positive due to janus kinase-3 deficiency718107000
- T-cell negative B-cell positive severe combined immunodeficiency due to JAK3 deficiency718107000
- Alymphoid cystic thymic dysgenesis720345008
- Congenital alopecia and nail dystrophy with severe functional T-cell immunodeficiency720345008
- Pignata Guarino syndrome720345008
- Severe T-cell immunodeficiency, congenital alopecia, nail dystrophy syndrome720345008
- T-cell immunodeficiency, congenital alopecia, and nail dystrophy720345008
- Winged helix deficiency720345008
- Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema syndrome720986005
- Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphoedema syndrome720986005
- OL-EDA-ID (anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema) syndrome720986005
- OL-EDA-ID (anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphoedema) syndrome720986005
- Primary immunodeficiency due to MCM4 (mini-chromosome maintenance complex component 4) deficiency724275005
- Primary immunodeficiency due to MCM4 deficiency724275005
- Primary immunodeficiency with natural killer cell deficiency and adrenal insufficiency724275005
- Combined immunodeficiency due to CD3-gamma deficiency725135004
- Combined immunodeficiency due to CD3gamma deficiency725135004
- Bare lymphocyte syndrome type 1725136003
- Bare lymphocyte syndrome type I725136003
- Immunodeficiency by defective expression of HLA (human leucocyte antigen) class 1725136003
- Immunodeficiency by defective expression of HLA (human leukocyte antigen) class 1725136003
- Immunodeficiency by defective expression of human leucocyte antigen class 1725136003
- Immunodeficiency by defective expression of human leucocyte antigen class I725136003
- Immunodeficiency by defective expression of human leukocyte antigen class 1725136003
- Immunodeficiency by defective expression of human leukocyte antigen class I725136003
- Immunodeficiency by defective expression of major histocompatibility complex class I725136003
- MHC (major histocompatibility complex) class I deficiency725136003
- Major histocompatibility complex class I deficiency725136003
- Combined immunodeficiency due to OX40 deficiency766879006
- Combined immunodeficiency with childhood-onset Kaposi sarcoma766879006
- Familial CD8 deficiency766983005
- Susceptibility to respiratory infection associated with CD8alpha chain mutation766983005
- Combined immunodeficiency due to STK4 (serine/threonine kinase 4) deficiency771479000
- Combined immunodeficiency due to STK4 deficiency771479000
- Combined immunodeficiency due to serine/threonine kinase 4 deficiency771479000
- Combined immunodeficiency due to MALT1 deficiency773488000
- Combined immunodeficiency due to mucosa-associated lymphoid tissue lymphoma translocation gene 1 deficiency773488000
- T-cell receptor alpha-beta-positive T-cell deficiency782750002
- TCR (T-cell receptor) alpha-beta-positive T-cell deficiency782750002
- Severe combined immunodeficiency due to IKK2 deficiency782751003
- Severe combined immunodeficiency due to inhibitor of nuclear factor kappa B kinase subunit beta deficiency782751003
- RIDDLE (radiosensitivity, immunodeficiency, dysmorphic features, learning difficulties) syndrome783099001
- RIDDLE syndrome783099001
- RNF168 (ring finger protein 168) deficiency783099001
- Radiosensitivity, immunodeficiency, dysmorphic features, learning difficulties syndrome783099001
- Combined immunodeficiency due to IL21R deficiency784340000
- Combined immunodeficiency due to interleukin 21 receptor deficiency784340000
- Lymphocyte count below reference range1156294003
- Progressive microcephaly, seizures, cortical blindness, developmental delay syndrome1172900005
- Severe combined immunodeficiency due to LAT (linker for activation of T cells) deficiency1179284005
- Severe combined immunodeficiency due to LAT deficiency1179284005
- Severe combined immunodeficiency due to linker for activation of T cells deficiency1179284005
- Combined immunodeficiency due to moesin deficiency1179285006
- MSN (moesin) related combined immunodeficiency1179285006
- X-linked moesin-associated immunodeficiency1179285006
- Combined immunodeficiency due to GINS complex subunit 1 deficiency1179286007
- Combined immunodeficiency due to GINS1 (GINS complex subunit 1) deficiency1179286007
- Combined immunodeficiency due to GINS1 deficiency1179286007
- Combined immunodeficiency with intrauterine growth retardation, NK (natural killer) cell deficiency, neutropenia1179286007
- Combined immunodeficiency due to TFRC deficiency1179288008
- Combined immunodeficiency due to transferrin receptor deficiency1179288008
- TFRC (transferrin receptor) related combined immunodeficiency1179288008
- B-cell expansion with NF-kB and T-cell anergy disease1179300002
- B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy disease1179300002
- BENTA (B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy) disease1179300002
- BENTA disease1179300002
- Combined immunodeficiency due to CARMIL2 deficiency1186712009
- Combined immunodeficiency due to RLTPR deficiency1186712009
- Combined immunodeficiency due to capping protein regulator and myosin 1 linker 2 deficiency1186712009
- Autosomal recessive lymphoproliferative disease due to ITK deficiency1186714005
- Combined immunodeficiency due to IL2 inducible T cell kinase deficiency1186714005
- Combined immunodeficiency due to ITK deficiency1186714005
- Combined immunodeficiency due to interleukin-2 inducible T cell kinase deficiency1186714005
- Combined immunodeficiency due to CD70 deficiency1186715006
- Combined immunodeficiency due to DOCK8 deficiency1197205005
- Combined immunodeficiency due to dedicator of cytokinesis 8 deficiency1197205005
- Combined immunodeficiency due to dedicator of cytokinesis 8 protein deficiency1197205005
- DOCK8 immunodeficiency syndrome1197205005
- Autosomal recessive combined immunodeficiency with multiple intestinal atresias1197428008
- CID-MIA/early-onset IBD - combined immunodeficiency-multiple intestinal atresia/early-onset inflammatory bowel disease1197428008
- Combined immunodeficiency due to TTC7A mutation1197428008
- Combined immunodeficiency, enteropathy spectrum1197428008
- Combined immunodeficiency due to LRBA deficiency1197477000
- Combined immunodeficiency due to lipopolysaccharide-responsive beige-like anchor protein deficiency1197477000
- NF-kappa-B-inducing kinase deficiency1197478005
- NIK deficiency1197478005
- Primary immunodeficiency with multifaceted aberrant lymphoid immunity1197478005
- DOCK2 deficiency1197479002
- Dedicator of cytokinesis 2 deficiency1197479002
- Severe combined immunodeficiency due to CORO1A deficiency1229942009
- Severe combined immunodeficiency due to coronin 1A deficiency1229942009
- T-B+ (T-cell negative B-cell positive) severe combined immunodeficiency1335927007
- T-B+ SCID - T-cell negative B-cell positive severe combined immunodeficiency1335927007
- T-cell negative B-cell positive severe combined immunodeficiency1335927007
- Combined immunodeficiency due to RELA haploinsufficiency1336027001
- Combined immunodeficiency due to FCH and mu domain containing endocytic adaptor 1 deficiency1340041000
- Combined immunodeficiency due to FCHO1 deficiency1340041000
- Developmental delay, immunodeficiency, leucoencephalopathy, hypohomocysteinemia syndrome1340174002
- Developmental delay, immunodeficiency, leukoencephalopathy, hypohomocysteinemia syndrome1340174002
- Autosomal dominant combined immunodeficiency due to AIOLOS deficiency1350218008
- Autosomal dominant combined immunodeficiency due to Aiolos deficiency1350218008
- Autosomal dominant combined immunodeficiency due to IKZF3 mutation1350218008
- Autosomal recessive CID (combined immunodeficiency) due to ICOS deficiency1351236000
- Autosomal recessive combined immunodeficiency due to ICOS deficiency1351236000
- Autosomal recessive combined immunodeficiency due to inducible T cell costimulator deficiency1351236000
- Autosomal recessive combined immunodeficiency due to ICOSLG deficiency1351245004
- Autosomal recessive combined immunodeficiency due to inducible T-cell costimulator ligand deficiency1351245004
- Autosomal dominant combined immunodeficiency due to ERBIN deficiency1351324009
- Autosomal dominant combined immunodeficiency due to erbb2 interacting protein deficiency1351324009
- Autosomal dominant hyperimmunoglobulin E syndrome due to ERBB2IP defect1351324009
- Autosomal dominant hyperimmunoglobulin E syndrome due to ERBIN deficiency1351324009
- Autosomal dominant combined immunodeficiency due to partial GP130 deficiency1351325005
- Autosomal dominant combined immunodeficiency due to partial IL6ST deficiency1351325005
- Autosomal dominant combined immunodeficiency due to partial glycoprotein 130 deficiency1351325005
- Autosomal dominant combined immunodeficiency due to partial interleukin 6 cytokine family signal transducer deficiency1351325005
- Autosomal dominant combined immunodeficiency due to partial interleukin 6 signal transducer protein deficiency1351325005
- Autosomal dominant hyperimmunoglobulin E syndrome due to partial IL6ST deficiency1351325005
- Autosomal recessive combined immunodeficiency due to IL6 receptor protein deficiency1351327002
- Autosomal recessive combined immunodeficiency due to IL6R deficiency1351327002
- Autosomal recessive combined immunodeficiency due to interleukin 6 receptor deficiency1351327002
- Autosomal recessive hyperimmunoglobulin E syndrome due to IL6R deficiency1351327002
- Autosomal recessive combined immunodeficiency due to GP130 deficiency1351328007
- Autosomal recessive combined immunodeficiency due to complete IL6 signal transducer protein deficiency1351328007
- Autosomal recessive combined immunodeficiency due to complete IL6ST deficiency1351328007
- Autosomal recessive combined immunodeficiency due to complete glycoprotein 130 deficiency1351328007
- Autosomal recessive combined immunodeficiency due to complete interleukin 6 cytokine family signal transducer deficiency1351328007
- Autosomal recessive hyperimmunoglobulin E syndrome due to complete IL6ST deficiency1351328007
- Stüve Wiedemann syndrome type 21351328007
- Autosomal recessive combined immunodeficiency due to partial GP130 deficiency1351329004
- Autosomal recessive combined immunodeficiency due to partial IL6 signal transducer protein deficiency1351329004
- Autosomal recessive combined immunodeficiency due to partial IL6ST deficiency1351329004
- Autosomal recessive combined immunodeficiency due to partial glycoprotein 130 deficiency1351329004
- Autosomal recessive combined immunodeficiency due to partial interleukin 6 cytokine family signal transducer deficiency1351329004
- Autosomal recessive hyperimmunoglobulin E syndrome due to partial IL6 signal transducer deficiency1351329004
- Autosomal recessive combined immunodeficiency due to DNA polymerase delta 1 catalytic subunit mutation1351332001
- Autosomal recessive combined immunodeficiency due to POLD1 mutation1351332001
- Autosomal recessive combined immunodeficiency due to DNA polymerase delta 2 accessory subunit mutation1351333006
- Autosomal recessive combined immunodeficiency due to POLD2 mutation1351333006
- Autosomal recessive DNA repair defect due to MCM10 deficiency1351341006
- Autosomal recessive combined immunodeficiency due to MCM10 deficiency1351341006
- Autosomal recessive combined immunodeficiency due to minichromosome maintenance complex component 10 deficiency1351341006
- Severe combined immunodeficiency due to BCL11 transcription factor B deficiency1351613000
- Severe combined immunodeficiency due to BCL11B deficiency1351613000
- Autosomal recessive combined immunodeficiency due to RELB deficiency1351650004
- Autosomal recessive combined immunodeficiency due to RELB mutation1351650004
- Autosomal recessive combined immunodeficiency due to RELB proto-oncogene, NF-kB subunit mutation1351650004
- Autosomal recessive combined immunodeficiency due to RelB mutation1351650004
- X-linked combined immunodeficiency due to SAM and SH3 domain containing 3 deficiency1351651000
- X-linked combined immunodeficiency due to SASH3 deficiency1351651000
- Autosomal recessive combined immunodeficiency due to COPG1 deficiency1351652007
- Autosomal recessive combined immunodeficiency due to COPI coat complex subunit gamma 1 deficiency1351652007
- Autosomal recessive combined immunodeficiency due to CD28 molecule mutation1351727000
- Autosomal recessive combined immunodeficiency due to CD28 mutation1351727000
- Autosomal recessive combined immunodeficiency due to WASP-interacting protein deficiency1351776006
- Autosomal recessive combined immunodeficiency due to WIP deficiency1351776006
- Autosomal recessive combined immunodeficiency due to WIPF1 mutation1351776006
- Autosomal recessive combined immunodeficiency due to Wiskott Aldrich syndrome protein-interacting protein deficiency1351776006
- Autosomal recessive combined immunodeficiency due to ARPC1B mutation1351778007
- Autosomal recessive combined immunodeficiency due to Arp2/3-mediated filament branching defect1351778007
- Autosomal dominant combined immunodeficiency due to STAT5b deficiency1351779004
- Autosomal dominant combined immunodeficiency due to STAT5b mutation1351779004
- Autosomal dominant combined immunodeficiency due to signal transducer and activator of transcription 5B mutation1351779004
- Autosomal recessive combined immunodeficiency due to REL mutation1351780001
- Autosomal recessive combined immunodeficiency due to REL proto-oncogene NF-kappa-B subunit mutation1351780001
- Autosomal recessive combined immunodeficiency due to c-Rel deficiency1351780001
- Autosomal recessive combined immunodeficiency due to BCL10 deficiency1351781002
- Autosomal recessive combined immunodeficiency due to BCL10 immune signaling adaptor mutation1351781002
- Autosomal recessive combined immunodeficiency due to BCL10 mutation1351781002
- Autosomal recessive combined immunodeficiency due to CHUK mutation1351800009
- Autosomal recessive combined immunodeficiency due to IKK-alpha deficiency1351800009
- Autosomal recessive combined immunodeficiency due to component of inhibitor of nuclear factor kappa B kinase complex mutation1351800009
- Combined immunodeficiency due to HELIOS deficiency1351801008
- Combined immunodeficiency due to IKAROS family zinc finger 2 mutation1351801008
- Combined immunodeficiency due to IKZF2 mutation1351801008
- Autosomal recessive combined immunodeficiency due to ITPKB deficiency1351802001
- Autosomal recessive combined immunodeficiency due to ITPKB mutation1351802001
- Autosomal recessive combined immunodeficiency due to inositol-trisphosphate 3-kinase B mutation1351802001
- Autosomal recessive combined immunodeficiency due to PAX1 deficiency1351804000
- Autosomal recessive combined immunodeficiency due to PAX1 mutation1351804000
- Autosomal recessive combined immunodeficiency due to paired box 1 mutation1351804000
- Combined immunodeficiency due to CARD11 deficiency1351892007
- Combined immunodeficiency due to caspase recruitment domain family member 11 protein deficiency1351892007
- Autosomal recessive combined immunodeficiency due to MAN2B2 deficiency1351946008
- Autosomal recessive combined immunodeficiency due to MAN2B2 mutation1351946008
- Autosomal recessive combined immunodeficiency due to mannosidase alpha class 2B member 2 mutation1351946008
- Autosomal recessive DNA repair defect due to POLE2 deficiency1354597009
- Autosomal recessive DNA repair defect due to polymerase-epsilon subunit 2 deficiency1354597009
- Autosomal recessive deoxyribonucleic acid repair defect due to DNA polymerase epsilon 2, accessory subunit deficiency1354597009
- Autosomal recessive DNA repair defect due to LIG1 deficiency1354649003
- Autosomal recessive DNA repair defect due to ligase 1 deficiency1354649003
- Autosomal recessive deoxyribonucleic acid repair defect due to DNA ligase 1 deficiency1354649003
- Progressive microcephaly, seizures, cortical blindness, developmental delay syndrome with combined immunodeficiency due to diaphanous related formin 1 mutation1354858008
- Progressive microcephaly, seizures, cortical blindness, developmental delay with combined immunodeficiency due to DIAPH1 deficiency1354858008
- Progressive microcephaly, seizures, cortical blindness, developmental delay with combined immunodeficiency due to DIAPH1 mutation1354858008
- SCBMS (seizures, cortical blindness, microcephaly syndrome) with combined immunodeficiency due to DIAPH1 mutation1354858008
- Autosomal recessive deoxyribonucleic acid repair defect due to NSMCE3 deficiency1354861009
- Autosomal recessive deoxyribonucleic acid repair defect due to NSMCE3 mutation1354861009
- DNA repair defect due to NSMCE3 deficiency1354861009
- LICS syndrome1354861009
- Lung disease, immunodeficiency, chromosome breakage syndrome1354861009
- Combined immunodeficiency due to FOXN1 haploinsufficiency1363572000
- Combined immunodeficiency due to forkhead box N1 haploinsufficiency1363572000
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.
Automate ICD-10 Coding With AI
Send clinical text to the AutoICD API and get back structured ICD-10 codes with confidence scores. Integrates into any EHR or billing system in minutes.
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.