Kutane T-Zell-Lymphome (CTCL) stellen eine heterogene Gruppe lymphoproliferativer Neoplasien dar.Nach der Klassifikation der EORTC (European Organization for Research and Treatment of Cancer ) werden die CTCL in 3 Kategorien eingeteilt:▂ indolente mit einer Überlebenszeit von >10 Jahren, ▂ aggressive mit einer Überlebenszeit von <10 Jahren sowie ▂ provisorisch eingeteilte Formen.Zu den indolenten Formen gehören die Mycosis fungoides mit/ohne follikulärer Muzinose, die pagetoide Retikulose, die lymphomatoide Papulose sowie das groß-zellige CD30-positive Lymphom. Die aggressive Gruppe umfasst das Sézary-Syndrom und das großzellige CD30-negative Lymphom, während elastolytisches Lym-
Abkürzungen
ADO ArbeitsgemeinschaftDermatologische 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"
Standard-und experimentelle Therapie kutaner T-Zell-Lymphomeretinoids 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.
AbstractCutaneous 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,