Abstract
A male infant with severe combined immunodeficiency (SCID) presented at 8 mos of age with failure to thrive and Staph pyoderma. Quant, immunoglobulins were: IgG 150 mg/dl; IgM 31 mg/dl and no detectable IgA. He had 20% complement receptor rosetting peripheral blood lymphocytes (PBL). Lymphocyte membrane immunofluorescence was positive only for IgM (18%). Total lymphocyte counts varied between 1200-2000/mm3. Antibodies (Abs) to tetanus and polio virus antigens were present. Following a submandibular salpingitis and a severe bilateral pneumonia, Abs to rubella virus were detected. Red cell ADA and NP were normal. Anergy to Candida and failure to sensitize to DNCB were demonstrated. Lymphocyte proliferative responses were absent to all mitogens and specific antigens but normal (SI ratios) to allogeneic cells in MLC. E-rosetting lymphocytes in the PB were very low, 3-8% (nl 50-65%). An increased proportion of cells bearing the HTLA marker, a T-cell surface antigen, was demonstrated after incubation of bone marrow cells (fraction 3) and PBL with bovine and human thymosin and upon thymic epithelial monolayers. However, these same thymic factors could not induce E-rosette formation or responsiveness to mitogens. The cells inducible for the HTLA T-cell marker with thymic factors may represent a precursor T-cell subpopulation responsible for the ontogenically primitive MLC response and perhaps play a role in the production of Abs.
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Ballow, M., Incefy, G., Good, R. et al. SEVERE COMBINED IMMUNODEFICIENCY WITH B LYMPHOCYTES, ANTIBODY SYNTHESIS AND RESPONSE TO ALLOGENEIC CELLS IN MLC. Pediatr Res 11, 484 (1977). https://doi.org/10.1203/00006450-197704000-00686
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DOI: https://doi.org/10.1203/00006450-197704000-00686
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