Selective T-cell deficiency in Turner's syndrome.
The case of a 29-year-old Caucasian woman with 45 X0 karyotype, known as Turner's syndrome, and a recently diagnosed selective T-cell deficiency is reported. The main clinical features of the patient were recurrent sinopulmonary infections and a negative skin test with seven common recall antigens. Laboratory findings included lymphocytopenia, highly elevated CD45RA/CD45R0 ratio, as well as reduced expression of the co-stimulatory molecules CD154, CD86, CD80 and CD28 on CD4+ cells in combination with disturbed lymphocyte transformation in vitro. Markedly decreased levels of interleukin (IL)-2R, both on lymphocyte surface as well as the soluble analog, suggest a new form of x-linked immunodeficiency associated with Turner's syndrome.
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Institute for Clinical Immunology, Friedrich-Schiller University, Jena, Germany.
Markert
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Vogelsang
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Jäger
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D000328:Adult; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics*; D008231:Lymphopenia / Q000209:etiology*; D013601:T-Lymphocytes / Q000276:immunology*; D014424:Turner Syndrome / Q000276:immunology*; D014960:X Chromosome