Abstract
In patients with severe combined immunodeficiency (i.e., lymphopenic agammaglobulinemia), both humoral and cell-mediated forms of immunity are lacking as a result of absence of immuno-blasts or lymphoid precursor cells capable of differentiating into plasma cells and small lymphocytes. The small lymphocytes of these infants do not respond to stimulation in vitro with phytohemagglutinin, allogeneic cells or antigens. This situation can be corrected by transplanting bone marrow from an HL-A compatible healthy donor. This finding establishes that the differentiative sites are intact. Macrophage function has not been evaluated in these patients. Nor is there clear evidence to indicate whether macrophages derive from the same defective precursor stem cells as lymphocytes. In these experiments, a glass-adherent cell population was obtained from two patients with severe combined immunodeficiency. After three days incubation, these adherent cells (i.e., macrophages) were added to highly purified lymphocytes from two PPD-sensitive donors. Such highly purified lymphocytes do not respond to antigens in the absence of at least one macrophage per 100 lymphocytes. The macrophages of our patients restored this response to PPD. In addition, a soluble factor (CMRF) collected from macrophage cultures of both patients, as described by Bach et al., also reconstituted the response of purified lymphocytes to PPD. These studies indicate that macrophages in severe combined immunodeficiency are capable of 1) restoring the response of purified lymphocytes to PPD and 2) synthesizing CMRF (conditioned medium reconstituting factor). They also confirm Bach et al.'s observation that CMRF can replace the macrophage in its interaction with purified lymphocytes.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Gatti, R., Good, R. Marcrophage function in severe combined immunodeficiency. Pediatr Res 5, 378–379 (1971). https://doi.org/10.1203/00006450-197108000-00033
Issue Date:
DOI: https://doi.org/10.1203/00006450-197108000-00033