The bare-lymphocyte syndrome: report on the registry.

The bare-lymphocyte syndrome is characterised by a combined immunodeficiency associated with the lack of expression of HLA A, B, and C antigens and the absence of beta 2 microglobulin on lymphocytes. Other antigens are present on the surface of lymphocytes. Nine case-reports have been collected by an international registry. Studies on these patients confirm the role of HLA molecules in the development of functional T lymphocytes.