Abstract
The syndrome of SCID represents a heterogenous group of congenital lethal disorders, characterized by defects of both T and B cell systems. The patient was an eight month old female who had normal numbers of B cells, T cells and T cell subsets but with marked defective humoral and cell-mediated immunity. Using a monoclonal antibody specific for gamma chain murine T cell receptor, we were able to identify a putative second T cell antigen receptor on this patient's peripheral blood mononuclear cells (PBM) which had been propagated in-vitro with Con A, OKT3 and recombinant IL2. This molecule was shown to be associated with the T3, antigen and was composed of two polypeptide chains with molecular weights of 56 kd and 41 kd. T cells of healthy individual propagated in a similar manner in-vitro were lacking this molecule. Investigation of pokeweed mitogen induced B lymphocyte differentiation in-vitro into Ig secreting cells was suggestive of intrinsic B cell dysfunction with normal T helper cell function and no evidence of increased suppressor cell activity. Following cytoreduction with Busulfan and Cyclophosphamide, bone marrow transplantation (BMT) was done using HLA incompatible lectin separated T lymphocyte depleted marrow from father, to correct this disorder. At 28 days post BMT there is early evidence of engraftment. These findings demonstrate further, heterogeneity of SCID.
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Pahwa, R., Platsoucas, C., Pahwa, S. et al. HETEROGENEITY OF SEVERE COMBINED IMMUNODEFICIENCY DISEASE (SCID); DEMONSTRATION OF PUTATIVE GAMMA CHAIN T CELL RECEPTOR. Pediatr Res 21 (Suppl 4), 316 (1987). https://doi.org/10.1203/00006450-198704010-00891
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DOI: https://doi.org/10.1203/00006450-198704010-00891