Demonstration of an Intrathymic Defect in a Case of Severe Combined Immunodeficiency Disease
- 28 August 1975
- journal article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 293 (9), 424-428
- https://doi.org/10.1056/nejm197508282930904
Abstract
An in vitro system has been developed to elucidate the nature of the cellular defect in primary immunodeficiency diseases. Incubation, on human thymic epithelial monolayer cultures, of peripheral blood lymphocytes and bone-marrow cells from a child with documented severe combined immunodeficiency disease resulted in the appearance of a population of cells that formed rosettes with sheep erythrocytes. The same cell preparation permitted the synthesis of antigen-specific, complement-dependent antibodies after in vitro education, as demonstrated in a plaque assay system. In addition, thymic tissue from the same child gave morphologic and functional evidence of maturation when cultured in vitro. The experimental results suggest that in this case, lymphoid precursor cells were present in the bone marrow but failed to differentiate to functional maturity due to a defect in maturation of thymic-tissue.(N Engl J Med 293:424–428,1975)Keywords
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