Successful bone marrow transplantation with split lymphoid chimerism in DiGeorge syndrome.

摘要: A female infant with DiGeorge syndrome associated severe T-cell immunodeficiency underwent a successful bone marrow transplantation from her HLA-identical, mixed leukocyte culture-nonreactive brother at 5 months of age. Mature circulating T cells and mitogen-induced proliferative responses were detectable 10 days posttransplant, by 8 posttransplant functional T- B-cell reconstitution was documented normal to mitogens levels serum immunoglobulins as well asin vitro andin vivo reactivity specific antigens production antibody cell-dependent antigensin vivo. Phytohemagglutinin-induced interleukin-2 cell surface receptor expression improved values observed posttransplant. Histologic examination appendix thoracic lymph node obtained 9 17 respectively, revealed near-normal lymphoid architecture, germinal center formation providing morphologic confirmation reconstitution. Stable split chimerism donor origin B remaining recipient in sex chromosome analysis. Two years the subject remains free serious infections. In conclusion, this case indicates that can produce peripheral immunoreconstitution without need for significant thymic influence, most likely source postthymic cells, should be considered therapeutic option patients deficiency.