Partial immunologic reconstitution and hyperimmunoglobulinemia-E in neonatally-acquired graft-versus-host disease

A three-month-old girl with congenital immune deficiency developed graft-versus-host disease following engraftment of maternal immunocompetent cells. T and B lymphocyte numbers increased and lymphocyte responsiveness to phytohemagglutinin normalized during the patient's hospitalization. However, these cells failed to respond to pokeweed mitogen and several specific antigens, suggesting that the expanding clone of alloreactive cells had limited immunologic potential. Serum IgE concentration rose from an undetectable level to 2,600 u/ml, indicating an immunoregulatory imbalance. HLA typing revealed that the patient's parents shared HLA antigen specificities. Finally, experimental administration of antithymocyte globulin had no beneficial effect upon the patient's clinical course or laboratory findings.