Lymphopenic immunologic deficiency in identical twins: lymphocyte allografting and graft-versus-host disease following treatment with albumin-gradient-separated paternal bone marrow cells.

The clinical, immunologic and pathologic features of the first recorded examples of lymphopenic immunologic deficiency in twins are presented. Eleven-month-old male identical twin infants were found to be severely lymphopenic and lacked demonstrable cell-mediated immunity and antibody formation. Each first-degree relative differed from the probands by one chromosome at the major human histocompatibility locus, HL-A. Because of their rapidly deteriorating clinical conditions, allogeneic bone marrow transplantation was undertaken. Circumvention of graft-versus-host disease was attempted by separation of donor bone marrow cells on a discontinuous-albumin-gradient and administration of only 5×10 immature nucleated marrow cells per kilogram infant body weight. Additionally, immunologic enhancement was attempted by pretreating the infants with human isoantisera to identifiable infant HL-A antigens not present in the marrow donor. Paternal lymphocyte allografting occurred, as demonstrated by lymphocyte cytotoxicity testing, but both infants succumbed to graft-versus-host disease at the end of 3 weeks.