Combined immune deficiency syndromes with primary T-cell defect and partial B-cell reactive hyperactivity. Immunological and morphological analysis of two unusual cases

Two cases of combined immunodeficiency with lymphopenia, thymic dysplasia, and defective immunoglobulin production are reported. Both show selective hypo-gammaglobulinemia (IgG and IgA respectively) and selective hyper-gammaglobulinemia (both IgE, IgA, and IgM respectively). The cases are classified...

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Veröffentlicht in:Virchows Archiv A Pathological Anatomy and Histology 1978-07, Vol.378 (4), p.329-340
Hauptverfasser: Konorza, G G, Krueger, G R, Evers, K G
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Sprache:eng
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Zusammenfassung:Two cases of combined immunodeficiency with lymphopenia, thymic dysplasia, and defective immunoglobulin production are reported. Both show selective hypo-gammaglobulinemia (IgG and IgA respectively) and selective hyper-gammaglobulinemia (both IgE, IgA, and IgM respectively). The cases are classified, by correlation of clinical and histopathological data as a variant of Fireman's disease.
ISSN:0340-1227
1432-2307
DOI:10.1007/BF00465599