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X-gebundene Agammaglobulinämie, chronisch-atrophische Gastritis und Adenokarzinom des Magens bei einem 15-jährigen Jungen

X-linked agammaglobulinemia, chronic atrophic gastritis, and adenocarcinoma of the stomach in a 15-year-old boy

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Zusammenfassung

Die X-gebundene Agammaglobulinämie (XLA) ist eine erbliche Erkrankung mit genetischen Defekten im Bereich der Bruton-Thyrosinkinase (Btk), die für die normale Ausreifung von B-Zellen eine entscheidende Rolle spielt. Wir berichten hier den extrem seltenen Fall eines 15-jährigen Jungen mit XLA, der eine chronisch-atrophische Gastritis und in der Folge ein Adenokarzinom des Magens entwickelte. Die Assoziation von XLA mit soliden Tumoren des oberen Gastrointestinaltrakts ist bislang bei Kindern weitestgehend unbekannt. Es wird empfohlen, alle Patienten mit XLA regelmäßig auf Zeichen einer chronisch-atrophischen Gastritis zu untersuchen, um etwaige bösartige Erkrankungen frühzeitig und vor deren Metastasierung diagnostizieren zu können.

Abstract

X-linked agammaglobulinemia (XLA) is a hereditary disorder with genetic defects of Bruton’s tyrosine kinase (Btk), which is essential for the normal differentiation of B cells. We report the unique case of a 15-year-old boy with XLA, who developed chronic atrophic gastritis and subsequent adenocarcinoma of the stomach. This association of disease entities is widely unknown in children today. We recommend screening all children with XLA for symptoms of chronic atrophic gastritis to ensure early diagnosis of possible malignancies prior to metastasis.

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Authors and Affiliations

  1. Kinderarztpraxis, Hamburg

    Gerd-Michael Lackmann

  2. Institut für Pathologie, Heinrich-Heine-Universität, Düsseldorf

    Christopher Poremba

  3. Kinderklinik, Uckermark-Klinik, Schwedt/Oder

    Volker Wahn

  4. Klinik für pädiatrische Hämatologie und Onkologie, Zentrum für Kinderheilkunde, Heinrich-Heine-Universität, Düsseldorf

    Tim Niehues

  5. Reinstorfweg 10a, 21107, Hamburg

    Gerd-Michael Lackmann

Authors
  1. Gerd-Michael Lackmann
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  2. Christopher Poremba
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  3. Volker Wahn
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  4. Tim Niehues
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Correspondence to Gerd-Michael Lackmann.

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Lackmann, GM., Poremba, C., Wahn, V. et al. X-gebundene Agammaglobulinämie, chronisch-atrophische Gastritis und Adenokarzinom des Magens bei einem 15-jährigen Jungen. Monatsschr Kinderheilkd 155 (Suppl 1), S6–S9 (2007). https://doi.org/10.1007/s00112-005-1122-5

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  • DOI: https://doi.org/10.1007/s00112-005-1122-5

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