FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term MHC class II deficiency ID (Ontology) DOID:5812 (Human Disease)
Definition A severe combined immunodeficiency that is characterized by deficiency of MHC class II molecules that causes lack of immune protection against bacteria, viruses, and fungi and thus causes early death in childhood, and has_material_basis_in autosomal recessive inheritance of mutation in the CIITA, RFX5, RFXANK, and RFXAP genes.
Also Known As "bare lymphocyte syndrome type II" ; "BLSII" ; "SCID due to absent class II HLA antigens"
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  combined immunodeficiency
   |__severe combined immunodeficiency
       |__MHC class II deficiency
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Is a severe combined immunodeficiency
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Synonyms
  • "bare lymphocyte syndrome type II" EXACT
    "BLSII" EXACT OMO:0003012
    "SCID due to absent class II HLA antigens" EXACT
Secondary IDs
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ICD10CM:D81.6
MESH:D016511
MIM:209920
NCI:C3895
SNOMEDCT_US_2023_03_01:71904008
UMLS_CUI:C0242583