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Langerhanszell-Histiozytose (LCH)

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Definition:
Heterogene inflammatorisch-proliferative Erkrankung, bei der es zur Granulombildung um pathologische Langerhanszell-Histiozyten kommt. 
Häufigkeit:
Selten. Die meisten Patient*innen sind Kinder.
Symptome:
Prinzipiell kann jedes Organsystem betroffen sein; am häufigsten Knochen, Haut, Lunge oder Zentralnervensystem.
Befunde:
Abhängig von der Lokalisation. U. a. Hautausschlag, Knochenschmerzen und Diabetes insipidus mit Polydipsie und Polyurie.
Diagnostik:
Bildgebende Verfahren und Biopsie mit Nachweis von Granulomen mit CD207+ (Langerin) Langerhanszell-Histiozyten. Ausführliche Diagnostik, um das Ausmaß des Organbefalls zu bestimmen.
Therapie:
Je nach Ausmaß abwartendes Beobachten, Lokaltherapie oder systemische Chemotherapie.
  • Gesellschaft für Pädiatrische Onkologie und Hämatologie: Langerhanszell-Histiozytose (LCH) im Kindes- und Jugendalter. AWMF-Registernummer 025-015. S1, Stand 2017. www.awmf.org 
  • Gesellschaft für Pädiatrische Radiologie: Ganzkörper-MRT im Kindesalter. S1-Leitlinie, AWMF-Registernummer 064-018. S1, Stand 2018. www.awmf.org 
  • Euro-Histio-Net : Referenznetzwerk für Langerhans-Zell-Histiozytose und assoziierte Syndrome
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  • Dietrich August, Dr. med., Arzt in Weiterbildung Innere Medizin, Freiburg