25. Immumolgical deficiency syndrome in nonidentical twins: attempts at treatment with transplanlation of bone marrow and fetal thymus

Abstract

This report describes nonidentical male twins with an immunedeficiency syndrome, which can not be classified into the known categories. The defect in cell-mediated immunity was characterized by lymphopenia, diminished response of lymphocytes to phytohemagglutinin (PHA) and allogeneic cells in culture, negative skin tests to various antigens, delayed rejection of a second set skin graft from a HLA-nonidentical donor. Humoral immunity was deficient in terms of diminshed production of IgG, IgA, and IgM, of isoagglutinins, and of antibody against poliovirus vaccine and tetanus toxoid. There were no plasma cells in the bone marrow. The children were kept within a sterile plastic isolator and maintained in a gnotobiotic state by antibiotic treatment from the early days of their life. In one child two thymus transplants induced a transient rise of peripheral lymphocyte count that a humoral factor of the thymus was operative. Since no cells of HLA type of the donor were found in the recipient. The second child received bone marrow cells from the mother separated in an albumin density gradient according to the method of Dicke and Van Bekknm. Fraction 3, containing 40 × 106 cells with a markedly reduced lymphocyte component was injected intravenously in this child treated before with ALG. There were no signs of secondary disease following transplantation. A transient rise of PHA response of lymphocytes, a temporarily positive skin test to DNCB, and an increased production of IgG were observed.