D81.6
BillableMajor histocompatibility complex class I deficiency
Major histocompatibility complex class I deficiency
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Bare lymphocyte syndrome
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) NOSM35.9
- •certain conditions originating in the perinatal periodP00-P96
- •complications of pregnancy, childbirth and the puerperiumO9A)O00
- •congenital malformations, deformations and chromosomal abnormalitiesQ00-Q99
- •endocrine, nutritional and metabolic diseasesE00-E88
- •human immunodeficiency virus [HIV] diseaseB20
- •injury, poisoning and certain other consequences of external causesS00-T88
- •neoplasmsC00-D49
- •symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classifiedR00-R94
Related Codes(9)
D81.0Severe combined immunodeficiency with reticular dysgenesis
D81.1Severe combined immunodeficiency w low T- and B-cell numbers
D81.2Severe combined immunodef w low or normal B-cell numbers
D81.3Adenosine deaminase [ADA] deficiency
D81.4Nezelof's syndrome
D81.5Purine nucleoside phosphorylase [PNP] deficiency
D81.7Major histocompatibility complex class II deficiency
D81.8Other combined immunodeficiencies
D81.9Combined immunodeficiency, unspecified
ICD-11 Equivalents(1)
ICD-11 Equivalents
View full mappingCorresponding ICD-11 codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(108)
SNOMED CT
- 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
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to beta-2-microglobulin mutation1351351007
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to beta-2-microglobulin mutation1351351007
- Immunodeficiency by defective expression of major histocompatibility complex class I due to beta-2-microglobulin mutation1351351007
- MHC (major histocompatibility complex) class I deficiency due to B2M mutation1351351007
- Major histocompatibility complex class I deficiency due to B2M mutation1351351007
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to transporter 1, ATP binding cassette subfamily B member mutation1351354004
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to transporter 1, ATP binding cassette subfamily B member mutation1351354004
- Immunodeficiency by defective expression of major histocompatibility complex class I due to transporter 1, ATP binding cassette subfamily B member mutation1351354004
- MHC (major histocompatibility complex) class I deficiency due to TAP1 mutation1351354004
- Major histocompatibility complex class I deficiency due to TAP1 mutation1351354004
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to transporter 2, ATP binding cassette subfamily B member mutation1351355003
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to transporter 2, ATP binding cassette subfamily B member mutation1351355003
- Immunodeficiency by defective expression of major histocompatibility complex class I due to transporter 2, ATP binding cassette subfamily B member mutation1351355003
- MHC (major histocompatibility complex) class I deficiency due to TAP2 mutation1351355003
- Major histocompatibility complex class I deficiency due to TAP2 mutation1351355003
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to TAP binding protein mutation1351356002
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to TAP binding protein mutation1351356002
- Immunodeficiency by defective expression of major histocompatibility complex class I due to TAP binding protein mutation1351356002
- MHC (major histocompatibility complex) class I deficiency due to TAPBP mutation1351356002
- Major histocompatibility complex class I deficiency due to TAPBP mutation1351356002
UMLS
- BARE LYMPHOCYTE SYNDROME, TYPE IC1858266
- BLS, TYPE IC1858266
- BLS, Type IC1858266
- Bare Lymphocyte Syndrome Type 1C1858266
- Bare Lymphocyte Syndrome, Type IC1858266
- Bare lymphocyte syndrome type 1C1858266
- Bare lymphocyte syndrome type IC1858266
- HLA CLASS I DEFICIENCY, 1C1858266
- HLA Class I DeficiencyC1858266
- HLA class I deficiencyC1858266
- Immunodeficiency by Defective Expression of MHC Class IC1858266
- Immunodeficiency by defective expression of HLA (human leucocyte antigen) class 1C1858266
- Immunodeficiency by defective expression of HLA (human leukocyte antigen) class 1C1858266
- Immunodeficiency by defective expression of human leucocyte antigen class 1C1858266
- Immunodeficiency by defective expression of human leucocyte antigen class IC1858266
- Immunodeficiency by defective expression of human leukocyte antigen class 1C1858266
- Immunodeficiency by defective expression of human leukocyte antigen class IC1858266
- Immunodeficiency by defective expression of major histocompatibility complex class IC1858266
- Immunodeficiency by defective expression of major histocompatibility complex class I (disorder)C1858266
- MHC (major histocompatibility complex) class I deficiencyC1858266
- MHC CLASS I DEFICIENCY 1C1858266
- MHC1D1C1858266
- Major histocompatibility complex class I deficiencyC1858266
- Bare Lymphocyte SyndromeC0242583
- Bare Lymphocyte SyndromesC0242583
- Bare lymphocyte syndromeC0242583
- Lymphocyte Syndrome, BareC0242583
- Lymphocyte Syndromes, BareC0242583
- Severe combined immunodeficiency due to absent class II human leukocyte antigensC0242583
- Syndrome, Bare LymphocyteC0242583
- Syndromes, Bare LymphocyteC0242583
- bare lymphocyte syndromeC0242583
Clinical Terms
- Bare lymphocyte syndrome
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to TAP binding protein mutation
- Immunodeficiency by defective expression of major histocompatibility complex class I
- MHC (major histocompatibility complex) class I deficiency due to B2M mutation
- Immunodeficiency by defective expression of human leucocyte antigen class 1
- Immunodeficiency by Defective Expression of MHC Class I
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to beta-2-microglobulin mutation
- Lymphocyte Syndromes, Bare
- Immunodeficiency by defective expression of human leucocyte antigen class I
- Bare lymphocyte syndrome type 1
- Syndromes, Bare Lymphocyte
- BLS, TYPE I
- Bare Lymphocyte Syndromes
- HLA class I deficiency
- Major histocompatibility complex class I deficiency due to B2M mutation
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to TAP binding protein mutation
- Immunodeficiency by defective expression of human leukocyte antigen class I
- Major histocompatibility complex class I deficiency due to TAP1 mutation
- Bare lymphocyte syndrome type I
- Immunodeficiency by defective expression of major histocompatibility complex class I due to transporter 1, ATP binding cassette subfamily B member mutation
- MHC (major histocompatibility complex) class I deficiency
- HLA CLASS I DEFICIENCY, 1
- MHC1D1
- Syndrome, Bare Lymphocyte
- Immunodeficiency by defective expression of human leukocyte antigen class 1
- MHC (major histocompatibility complex) class I deficiency due to TAP1 mutation
- Immunodeficiency by defective expression of major histocompatibility complex class I due to beta-2-microglobulin mutation
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to transporter 2, ATP binding cassette subfamily B member mutation
- MHC (major histocompatibility complex) class I deficiency due to TAPBP mutation
- MHC CLASS I DEFICIENCY 1
- Immunodeficiency by defective expression of major histocompatibility complex class I due to TAP binding protein mutation
- Immunodeficiency by defective expression of HLA (human leukocyte antigen) class 1
- Immunodeficiency by defective expression of HLA (human leucocyte antigen) class 1
- Major histocompatibility complex class I deficiency due to TAP2 mutation
- Bare Lymphocyte Syndrome, Type I
- Severe combined immunodeficiency due to absent class II human leukocyte antigens
- Immunodeficiency by defective expression of major histocompatibility complex class I (disorder)
- MHC (major histocompatibility complex) class I deficiency due to TAP2 mutation
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to transporter 1, ATP binding cassette subfamily B member mutation
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to transporter 1, ATP binding cassette subfamily B member mutation
- Major histocompatibility complex class I deficiency due to TAPBP mutation
- Immunodeficiency by defective expression of human leucocyte antigen class 1 due to transporter 2, ATP binding cassette subfamily B member mutation
- Immunodeficiency by defective expression of major histocompatibility complex class I due to transporter 2, ATP binding cassette subfamily B member mutation
- Lymphocyte Syndrome, Bare
- Immunodeficiency by defective expression of human leukocyte antigen class 1 due to beta-2-microglobulin mutation
Frequently Asked Questions
What is the ICD-10 code for major histocompatibility complex class i deficiency?
The ICD-10-CM code for major histocompatibility complex class i deficiency is D81.6. The full clinical description is "Major histocompatibility complex class I deficiency". D81.6 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code D81.6 mean?
ICD-10-CM code D81.6 represents “Major histocompatibility complex class I deficiency”. 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 D81.6 a billable code?
Yes, D81.6 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is D81.6 in?
D81.6 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).
What codes cannot be used with D81.6?
D81.6 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 D81.6 map to?
D81.6 maps to 5 SNOMED CT concepts: 725136003, 1351356002, 1351351007, 1351354004, 1351355003. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for D81.6?
D81.6 is linked to 2 UMLS Concept Unique Identifiers: C1858266, C0242583. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does D81.6 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like major histocompatibility complex class i deficiency 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 D81.6?
D81.6 maps to the ICD-11 code: 4A01.11 (Major histocompatibility complex class I deficiency).
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.