Virchows Archiv A

, Volume 378, Issue 4, pp 329–340 | Cite as

Combined immune deficiency syndromes with primary T-cell defect and partial B-cell reactive hyperactivity

Immunological and morphological analysis of two unusual cases
  • G. G. Konorza
  • G. R. F. Krueger
  • K. G. Evers
Article

Summary

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.

Key words

Immune deficiency disease Fireman's disease Pathology Clinical course 

Zusammenfassung

Es werden zwei FÄlle von kombiniertem Immunmangelsyndrom beschrieben mit Lymphopenie, Thymusdysplasie and fehlerhafter Immunglobulinbildung. Beide zeigen eine selektive Hypo-GammaglobulinÄmie (IgG bzw. IgA) und selektive Hyper-GammaglobulinÄmie (beide IgE, IgA bzw. IgM). Die Klassifikation erfolgt durch eingehende Korrelation klinischer und pathologisch-anatomischer Daten als Variante der Firemanschen Erkrankungen.

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Copyright information

© Springer-Verlag 1978

Authors and Affiliations

  • G. G. Konorza
    • 1
    • 2
  • G. R. F. Krueger
    • 1
    • 2
  • K. G. Evers
    • 1
    • 2
  1. 1.Immunopathology Laboratories, Pathology InstituteUniversity of CologneGermany
  2. 2.Children's ClinicCity of Cologne

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