Hemiallogeneic bone marrow transplantation in a child with severe combined immunodeficiency disease

Immunologic reconstitution of a 16-month-old male infant with severe combined immunodeficiency disease was attempted using maternal bone marrow cells in the absence of an HL-A-identical donor. Following transplantation, lymphocytes from the recipient became responsive to phytohemagglutinin, Candida albicans, and type 12 M-protein from group A beta hemolytic streptococci. Karyotyping following phytohemagglutinin stimulation revealed that one fifth the responsive cells were of recipient origin (XY). Early signs of graft-versus-host reaction were noted by the fourteenth day. The involvement of skin, lungs, liver, and spleen became worse during the ensuing week, but by the twenty-first day, the patient's condition began to improve. The use of methotrexate, bowel sterilization, maternal plasma infusions, and other supportive measures were thought to be responsible for mitigating the graft-versus-host reaction. Despite substantial clearing of the involvement of the lungs, liver, and spleen, the patient died 41 days after transplantation.