Abstract
Fatal graft-versus-host (G-V-H) disease has been an invariable accompaniment of incompatible bone marrow transplantation in infants with lymphopenic defects. This report describes an approach which permitted circumvention of this complication. Histocompatibility studies demonstrated no HL-A compatible donors for a female infant with lymphopenic agammaglobulinemia (very low IgG, absent IgA, IgM, and IgE). Serum from her mother (para 16) contained antibodies (1:64 titer) to paternal HL-A antigens inherited by the patient. Maternal lymphocytes were not stimulated by paternal or infant's leukocytes in mixed leukocyte culture in media containing maternal plasma. Immunologic enhancement was attempted at age 5 months by pretreating the infant with cell-free maternal plasma I.V. 24 hours before the I.V. infusion of 5 × 106 immature albumin-gradient-separated maternal marrow cells. Appetite and growth increased dramatically during the first 3 months, thrush and pneumonia cleared, and no overt signs of G-V-H were noted. Delayed cutaneous responsiveness to C. albicans was seen by the 13th day and persists. Lymph node biopsy at 90 days contained plasma cells which stained with fluoroscein-conjugated anti-IgG, IgA, IgM or IgE. A second maternal marrow transplant was given at age 11 months, again without overt G-V-H. This 19-month-old infant has gained 4 kg and grown 20 cm since the first transplant 14 months ago. A normal serum IgA concentration and somewhat low but constant levels of IgG and IgM are present now 5 months after the last plasma infusion. These findings suggest possible usefulness of immunologic enhancement in future attempts when compatible donors are unavailable.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Buckley, R., Amos, D., Kremer, W. et al. Incompatible bone marrow transplantation in lymphopenic immunologic deficiency. Pediatr Res 5, 379 (1971). https://doi.org/10.1203/00006450-197108000-00034
Issue Date:
DOI: https://doi.org/10.1203/00006450-197108000-00034