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Immundefekt und andere parathymische Syndrome bei Thymom

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Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics

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Zusammenfassung

Im Jahre 1954 erwähnte Good [1] erstmals einen Patienten, der die ungewöhnliche Kombination eines Thymustumors mit einer Hypogammaglobulinämie aufwies. Er maß diesem Zusammentreffen zweier an sich seltener Befunde eine besondere Bedeutung zu, da die Beobachtung in eine Zeit fiel, in der — unter anderem auch durch Good und seine Mitarbeiter — grundlegende immunologische Erkenntnisse erarbeitet und verschiedene Formen angeborener Immundefekte charakterisiert wurden. Goods Patient litt seit dem Alter von 54 Jahren unter chronischen Luftwegsinfekten mit rezidivierenden Pneumonien sowie an Müdigkeit und markantem Gewichtsverlust. Bei der Untersuchung wurde ein spindelzelliges Thymom entdeckt und operativ entfernt; die Infektanfälligkeit verstärkte sich jedoch in den folgenden 3 Jahren. Serologische Untersuchungen zeigten eine ausgeprägte Hypogammaglobulinämie und eine stark verminderte Antikörperproduktion nach aktiver Immunisierung mit verschiedenen Impfstoffen. Auch Hauttests vom Spättyp fielen negativ aus. Zusätzlich zu diesen immunologischen Defekten fand man eine aplastische Anämie [2], Nach verschiedenen septischen Episoden verstarb der Patient im Alter von 58 Jahren an einer fulminanten, wahrscheinlich viralen Hepatitis.

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  1. Institut für klinisch-experimentelle Tumorforschung, Universität Bern und Medizinische Klinik, Tiefenauspital, CH-3004, Bern, Switzerland

    H. Keller

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  1. A. Morell
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P. Frick G.-A. von Harnack K. Kochsiek G. A. Martini A. Prader

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Morell, A., Keller, H. (1998). Immundefekt und andere parathymische Syndrome bei Thymom. In: Frick, P., von Harnack, GA., Kochsiek, K., Martini, G.A., Prader, A. (eds) Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics. Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics, vol 57. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-73558-5_6

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