Abstract
SCID is a heterogeneous disorder often associated with lymphopenia; hypothesized to be due to defective lymphoid differentiation or proliferation. We studied this hypothesis by analyzing BM mononuclear cells from 6 pt with SCID (as well as 5 age matched controls) for the presence of lymphoid progenitor cells. Such cells are E rosette , surface Ig and include cells that express terminal transferase (TDT), the common ALL antigen (CALLA) or p24. BM cells were studied using immunofluorescent microscopy. Over 1000 cells per slide were examined and counted. Two patterns were seen. Group 1 (n=2) with no detectable lymphoid progenitor cells were more lymphopenic (p= .03) than pt in group 2 (n=4). Both groups were more lymphopenic than age matched controls (p=.04). All pt had SCID based on absent response to mitogens, recurrent infections and failure to thrive. 5/6 pt had hypogammaglobulinemia. Group 2 contained 1 pt with adenosine deaminase deficiency on transfusion therapy and 1 pt with the spurious lymphocyte syndrome. These results suggest that some pts with SCID have reduced numbers of BM lymphoid precursors and the absence of such cells may be related to the severity of lymphopenia seen.
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Neudorf, S., Kersey, J. & Filipovich, A. BONE MARROW (BM) LYMPHOID PROGENITOR CELLS IN SEVERE COMBINED IMMUNODEFICIENCY (SCID). Pediatr Res 18 (Suppl 4), 261 (1984). https://doi.org/10.1203/00006450-198404001-01009
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DOI: https://doi.org/10.1203/00006450-198404001-01009