Severe candidiasis associated with thymic dysplasia, IgA deficiency, and plasma antilyMPHOCYTE EFFECTS

Evidences of impaired cell-mediated immunity were found in a boy having mucocutaneous candidiasis and chronic pulmonary disease. The thymus gland and peripheral lymphatic tissues were dysplastic. IgA was absent from serum and secretions. The patient's plasma clumped leucocytes in suspension, an...

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Veröffentlicht in:Pediatrics (Evanston) 1970-06, Vol.45 (6), p.926-936
Hauptverfasser: Schlegel, R J, Bernier, G M, Bellanti, J A, Maybee, D A, Osborne, G B, Stewart, J L, Pearlman, D S, Ouelette, J, Biehusen, F C
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Sprache:eng
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Zusammenfassung:Evidences of impaired cell-mediated immunity were found in a boy having mucocutaneous candidiasis and chronic pulmonary disease. The thymus gland and peripheral lymphatic tissues were dysplastic. IgA was absent from serum and secretions. The patient's plasma clumped leucocytes in suspension, an effect enhanced by bovida antigens. In vitro lymphocyte transformation, proliferation, and replication were normal in systems free of patient plasma but were partially inhibited by his plasma and totally inhibited by his plasma and bovida antigens. Large amounts of antibovida antibodies were found in the patient's plasma; and, when they were removed, lymphocyte functions were partially restored, even in the presence of bovida antigens. A plasma lymphocvtotoxic assay was positive. This appears to be a distinct syndrome comprising thymic dysplasia, IgA deficiency, antibovida antibodies, various types of plasma-mediated antilymphocyte effects, defective cellular and humoral immunity, and severe candidiasis.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.45.6.926