/MHC class I deficiency

MHC class I deficiency

Rare Disease

MONDO:0011476

Also Known As

BLS, type IBLSIBare lymphocyte syndrome type 1HLA CLASS I deficiencybare lymphocyte syndrome type Iimmunodeficiency by defective expression of HLA class 1immunodeficiency by defective expression of HLA class type 1BARE lymphocyte syndrome, type IBLS type 1Bare lymphocyte syndrome, type 1Bls, type 1HLA Class 1 deficiency

Definition

Immunodeficiency by defective expression of HLA class 1 is a very rare, primary, genetic, immunodeficiency disorder characterized by partial or complete absence of human leukocyte antigen class I expression resulting in a non-specific clinical picture of impaired immune response and susceptibility to infections.