Zusammenfassung
Die Histiozytosesyndrome stellen eine heterogene Gruppe von Krankheiten dar, bei der mononukleäre phagozytierende bzw. dendritische Zellen das Gewebe infiltrieren. Durch Proliferation von normalerweise akzessorischen Zellen mit antigenprozessierender oder antigenpräsentierender Funktion, die ubiquitär im Körper vorkommen, finden sich Krankheitsbilder mit sehr unterschiedlichen Symptomen. Neben organspezifischen Dysfunktionen durch Organinfiltration bzw. -destruktion können auch Störungen der Immunregulation im Vordergrund stehen. Zu den charakteristischen Symptomen gehören Hautveränderungen, Knochendestruktion, Hepatosplenomegalie, endokrinologische Ausfälle, neurologische Defizite sowie Zeichen der Knochenmarkinsuffizienz.
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Wessalowski, R., Lehrnbecher, T., Janka, G. (2014). Histiozytosesyndrome. In: Reinhardt, D., Nicolai, T., Zimmer, KP. (eds) Therapie der Krankheiten im Kindes- und Jugendalter. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-41814-3_57
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