Abstract
Extract: A nine-month-old male with recurrent pulmonary and gastrointestinal infections and persistent lymphopenia was presumed to have hereditary thymic dysplasia. He was unresponsive to intradermal injections of Monilia antigen, Varidase®, and diphtheria toxoid, and could not be sensitized to dinitro fluorbenzene. He failed to reject a skin allograft. Peripheral blood lymphocytes were unresponsive to phytohemagglutinin stimulation. The serum contained markedly diminished concentrations of γA, γM, and γG globulins, and he could not form specific antibodies to a variety of injected antigens.
A female sibling of the child was presumed to have leukocytes that shared the major histocompatibility antigens with the leukocytes of her brother. Consequently, 100 × 106 bone marrow cells were transplanted from the sister into the affected infant. Five days later, what appeared to be a graft-versus-host reaction was noted with hepatosplenomegaly, a maculopapular rash, edema, diarrhea, and, terminally, profuse hemorrhage. At autopsy the presumptive diagnosis was confirmed: a rudimentary thymus gland weighing 1.5 g was found. Lymph nodes and spleen lacked lymphoid follicles. Plasmacytoid cells, presumably of donor origin, were found in great abundance in the spleen, blood, and bone marrow.
Speculation: Further attempts to reconstitute immunocompetence in infants with thymic dysplasia should be pursued with transplants of histocompatible tissues. The limitations of current methodology in assessing minor differences in histocompatibility suggest, however, that immunosuppressive therapy is required to prevent the lethal effects of seemingly histocompatible cells from an immunocompetent donor in an immunologically unresponsive host. Ideally, such transplants should be performed only when a donor identical with the recipient at the HL-A locus is available.
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Kretschmer, R., Jeannet, M., Mereu, T. et al. Hereditary Thymic Dysplasia: A Graft-Versus-Host Reaction Induced by Bone Marrow Cells with a Partial 4a Series Histoincompatibility. Pediatr Res 3, 34–40 (1969). https://doi.org/10.1203/00006450-196901000-00005
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DOI: https://doi.org/10.1203/00006450-196901000-00005
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