Abstract
A boy with severe combined immunodeficiency received bone marrow transplantation from an HLA-compatible sibling, and immunological reconstitution was achieved. He had hypogammaglobulinemia, decrease in T cell number, inbalance of T cell subsets, and defective lymphocyte blastoid transformation. At age of 14 months, bone marrow cells from his sister were infused intravenously. Eleven days after the transplantation, positive mitogenic responses to PHA and Con A were first demonstrated. However, acute graft-versus-host disease was observed at 2 weeks, but it was mild and lasted for a few weeks. Two months later, serum IgG and IgM increased rapidly up to the levels of 888 mg/dl and 268 mg/dl, respectively, accompanied by the elevation of specific serum antibody titers, and the number of T cells and T cell subsets were normalized. Positive delayed-type cutaneous hypersensitivities to some specific antigens were also demonstrated. Lymphocyte chimerism after marrow transplantation was shown by analysis of sex-chromosome. He has been well, for the last 21 months without any treatment.
