Abstract
The Bare Lymphocyte Syndrome (BLS) is a rare immunodeficiency in which surface expression of HLA-DR antigens is absent. In the absence of expression of Major Histocompatibility Complex (MHC) there is an abnormal interaction between immunocytes with a resultant combined immunodeficiency. We report here of a 4 year old female with the BLS. This child is agammaglobulinemic and has poor cell mediated immunity with recurrent bacterial and viral infections. The child persistently excretes the polio vaccine virus type II in the stool. In vitro lymphocyte studies revealed normal responses to phytohemagglutinin and in the one-way mixed lymphocyte culture. Mitomycin C treated patient's cells were, however, unable to stimulate allogeneic lymphocytes, and the patient's lymphocytes did not respond to antigenic stimuli. In vitro treatment of patient's lymphocytes with α or γ interferon restored the expression of the MHC and improved antigenic and allogeneic responses. The patient's lymphocytes did not, however, secrete α or γ interferon. It is postulated that in this instance the BLS may be due to a primary interferonopathy.
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Krieger, B., Calvelli, T., Kadish, A. et al. 1042 INTERFERONOPATHY IN THE BARE LYMPHOCYTE SYNDROME. Pediatr Res 19, 284 (1985). https://doi.org/10.1203/00006450-198504000-01072
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DOI: https://doi.org/10.1203/00006450-198504000-01072
