Kutane Lymphome

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DGIM Innere Medizin

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Zusammenfassung

Hautlymphome umfassen ein breites Spektrum an prognostisch unterschiedlichen Subtypen, wobei T-Zell-Lymphome die häufigste Untergruppe darstellen. Für die endgültige Diagnosestellung ist eine sorgfältige klinisch-pathologische Korrelation entscheidend. Das häufigste Hautlymphom stellt die Mycosis fungoides dar, welche in Abhängigkeit des Krankheitsstadiums durch Patches, Plaques, Tumoren oder eine universelle Rötung des Hautorgans (= Erythrodermie) gekennzeichnet ist. Während in frühen Stadien bei der Mycosis fungoides meist hautgerichtete Therapiemaßnahmen ausreichen, sind in fortgeschrittenen Stadien und beim Sézary-Syndrom Systemtherapien oder multimodale und interdisziplinäre Therapiekonzepte indiziert. Zu den kutanen T-Zell-Lymphomen zählen des Weiteren indolente Subtypen mit meist solitärer Hautmanifestation wie die CD4-positive Lymphoproliferation oder das akrale CD8-positive Lymphom, zum anderen jedoch auch seltene aggressive Entitäten wie das periphere T-Zell-Lymphom oder das aggressive epidermotrope T-Zell-Lymphom. Kutane B-Zell-Lymphome sind selten. Während die Lebenserwartung beim kutanen Marginalzonenlymphom und kutanen follikulären B-Zell-Lymphom – trotz häufig kutaner Rezidive im Krankheitsverlauf – nahezu unbeeinträchtigt ist, zeigt das kutane großzellige B-Zell-Lymphom ein aggressives biologisches Verhalten.

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Abbreviations

R-CHOP:

Rituximab, Cyclophosphamid, Doxorubicin, Vincristin, Predniso(lo)n

WHO:

World Health Organization

EORTC:

European Organization for Research and Treatment of Cancer

CLA:

kutanes Lymphozytenantigen

CCR4:

C-C-Chemokinrezeptor Typ 4

NFkB:

nuclear factor kappa B

JAK:

Januskinase

STAT:

signal transducers and activators of transcription

MAPK:

mitogen-activated protein kinase

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Wobser, M. (2023). Kutane Lymphome. In: Lehnert, H., Märker-Hermann, E., Marx, N., Meyhöfer, S.M. (eds) DGIM Innere Medizin. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54676-1_514-2

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  • DOI: https://doi.org/10.1007/978-3-642-54676-1_514-2

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Chapter history

  1. Latest

    Kutane Lymphome
    Published:
    19 April 2023

    DOI: https://doi.org/10.1007/978-3-642-54676-1_514-2

  2. Original

    Primär kutane Lymphome
    Published:
    06 June 2015

    DOI: https://doi.org/10.1007/978-3-642-54676-1_514-1

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