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Standard- und experimentelle Therapie kutaner T-Zell-Lymphome

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Zusammenfassung

Kutane T-Zell-Lymphome umfassen eine Gruppe von Krankheiten mit dem Merkmal einer Invasion von monoklonalen T-Lymphozyten in die Haut. Aufgrund klinischer, histologischer und immunhistochemischer Merkmale können die kutanen T-Zell-Lymphome in 3 Gruppen eingeteilt werden: Indolente mit einer Überlebenszeit von >10 Jahren, aggressive mit einer Überlebenszeit von <10 Jahren sowie provisorisch eingeteilte Formen (EORTC-Klassifikation). Herkömmliche Therapien wie PUVA, Ganzhautbestrahlung mit schnellen Elektronen, Methotrexat, Polychemotherapie, Retinoiden sowie Photopherese werden seit vielen Jahren eingesetzt. Neu zugelassen ist Bexaroten. Im Streben nach besseren Ansprechraten werden im Rahmen von Studien neue Medikamente wie Imiquimod, Denileukin-diftitox, liposomales Doxorubicin und Adeno-Interferon-γ sowie Kombinationen der verschiedenen Therapiemodalitäten untersucht.

Abstract

Cutaneous T-cell lymphoma represent a heterogeneous group of diseases characterized by skin invasion of monoclonal T-lymphocytes. These cutaneous T-cell lymphomas are divided into 3 groups based on clinical, histological and immunohistological characteristics: Indolent with a survival time of over 10 years, aggressive with a survival time less than 10 years and provisional (EORTC classification). Standard treatments such as PUVA, total skin electron beam, methotrexate, polychemotherapy regimens, retinoids and photopheresis have been used for years. Bexarotene is a newly registered drug. To achieve better response rates, several new drugs are being evaluated in clinical trails, including imiquimod, denileukon-diftitox, liposomal doxorubicin, adeno-interferon-γ and various combination approaches.

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Abbreviations

ADO:

Arbeitsgemeinschaft Dermatologische Onkologie

CBCL:

kutane B-Zell-Lymphome

CTCL:

kutane T-Zell-Lymphome

EORTC:

European Organization for Research and Treatment of Cancer

FDA:

Food and Drug Administration

IFN:

Interferon

IL:

Interleukin

MHC:

„major histocompatibility complex“

PUVA:

Psoralen und UVA-Behandlung

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Beyeler, M., Dummer, R. Standard- und experimentelle Therapie kutaner T-Zell-Lymphome. Hautarzt 54, 1177–1184 (2003). https://doi.org/10.1007/s00105-003-0630-2

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