Abstract
The development of immunoglobulin production in a child with aucosomal recessive ADA+ SCID after therapy with cultured thymus epithelial cells is reported. The child is unique because she had not received gamma globulin or plasma prior to transplant while in laminar flow isolation. Transplacental IgG decreased to 2.7 mg/dl at 9 months of age, or 28 days post-transplant (PT). A steady increase of IgG began at 37 days PT, with IgA appearing at 70 days as determined by immunoelectrophoresis (IEP) and radial immunodiffusion (RID). IgM appeared 12 weeks PT on the surface of lymphocytes, 7 days prior to its detection in the serum. Five months after therapy IgG was 2030 mg/dl, IgM was 29 mg/dl, IgA was 55 mg/dl and IgA in lacrlmal fluid was 3.2 mg/dl. Initial IEP patterns with polyvalent antisera to IgG had 3 distinct overlapping arcs, gamma chain had one arc, kappa had 2 arcs and lambda and alpha each had one arc. Kappa chains were in excess over lambda. Repeated IEP over 3 months continue to show multiple arcs but IgG arcs are now joined and IgM is present. The “reversed” order of appearance of IgG and IgM may be related to: a) the underlying defect; b) lack of antigenic stimulation during the first year of life; or c) this form of immune therapy. The joining of the multiple precipitin lines may represent normal development of immunoglobulin. This case stresses the necessity for thymus function in immunoglobulin production in SCID patients.
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Jones, J., Sieber, O., Pinnas, J. et al. IMMUNOGLOBULIN PRODUCTION IN SEVERE COMBINED IMMUNODEFICIENCY DISEASE (SCID) AFTER THYMUS EPITHELIAL EXPLANT THERAPY. Pediatr Res 11, 488 (1977). https://doi.org/10.1203/00006450-197704000-00710
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DOI: https://doi.org/10.1203/00006450-197704000-00710