Transplantation · 1978
Successful nonsibling bone marrow transplantation in severe combined immunodeficiency.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Ramsøe K, Skinhøj P, Andersen V, Ernst P, Faber V, Platz P, Thomsen M, Svejgaard A, Eriksen K R, Plesner T, Morling N, Philip J, Killmann S A, Koch C, Muller-Bérat N, Henningsen K, Axelsen H
Аннотация
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.