The bare lymphocyte syndrome.
The bare lymphocyte syndrome is a combined immunodeficiency resulting from the lack of expression of either class I or class II HLA antigens at the cell surface. The main clinical manifestations are infections of the respiratory or the digestive tract. The immunodeficiency involves the absence of antibody formation and the absence of cell-mediated response, to specific antigen, contrasting with virtually normal transplant immunity to allogeneic determinants. The responsible gene(s) is not born by chromosome 6. The best treatment appears, at the present time, to be in utero stem cell transplantation into the sick fetus, and it may, in the future, be gene therapy.
Touraine
J L
JL
Department of Transplantation and Clinical Immunology, Claude Bernard University, Lyon, France.
Marseglia
G L
GL
Betuel
H
H
Souillet
G
G
Gebuhrer
L
L
D002675:Child, Preschool; D005260:Female; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000628:therapy; D007223:Infant; D008214:Lymphocytes / Q000276:immunology*; D008297:Male
D006680:HLA Antigens