K Ramsøe, P Skinhøj, V Andersen, P Ernst, V Faber, P Platz, M Thomsen, A Svejgaard, K R Eriksen, T Plesner, N Morling, J Philip, S A Killmann, C Koch, N Muller-Bérat, K Henningsen, H Axelsen
Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.
|Number of pages||3|
|Publication status||Published - 1978|
Keywords: Bone Marrow Transplantation; Female; HLA Antigens; Humans; Immunologic Deficiency Syndromes; Infant; Male; Pedigree; Transplantation, Homologous