Primäre kutane T-Zell Lymphome (CTCL) sind gekennzeichnet durch einen Tropismus maligner T-Zellen in das Hautorgan und besonders die Epidermis. Die klassischen und häufigsten Formen des CTCL stellen die Mycosis fungoides (MF) und das Sézary-Syndrom (SS). Der Verlauf der Erkrankung ist zu Beginn über viele Jahre indolent, jedoch ist insgesamt eine Progression der Erkrankung mit sukzessivem Befall der Lymphknoten, des Blutes oder der viszeralen Organe zu erwarten. Bei Patienten in den frühen Stadien IA, IB (T1, T2) liegt die 10-Jahres-Überlebensrate zwischen 100 und 67,4 % [1]
[2]. Entwickelt sich jedoch eine Transformation des Tumors von einer kleinzelligen in eine großzellige Variante, sinkt die mittlere Lebenserwartung auf 19,4 Monate; sind die Rezirkulationsorgane Lymphknoten (mittlere Lebenserwartung = 4,2 Jahre), Blut oder viszerale Organe (2-Jahres-Überlebensrate = 66 %) befallen, ist die Lebenserwartung deutlich erniedrigt. Häufig führen initial eingeleitete Standardtherapiemaßnahmen zu Remissionen, jedoch kommt es in der Regel zu Rezidiven. Die Therapie bleibt somit palliativ. Im Folgenden wird das Standardvorgehen bei der Behandlung von kutanen T-Zell-Lymphomen dargelegt, das z. B. die Photochemotherapie, Interferone und Retinoide umfasst; darüber hinaus werden neue Therapieoptionen diskutiert.
Abstract
Primary cutaneous T-cell lymphomas (CTCL) are characterized by homing of malignant T-cells into the skin and especially into the epidermis. The classical and most common forms of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS). The course of the disease is usually indolent for several years; progression of the disease, however, occurs slowly in every patient and involves the recirculation compartments, i. e. lymph nodes and peripheral blood, and finally spreads to the visceral organs. Early stage MF patients (stage IA/IB) have a 10-year survival rate of about 100 %/67 % [1]. Patients, however, who undergo transformation to large-cell lymphoma have a poor prognosis (medium time of survival: 19.4 months). Once lymph nodes (medium time of survival: 4.2 years) or blood and visceral organs (2-year survival rate: 66 %) are involved the survival rate also drops consideraly. Although there is an initial response to standard therapy, all patients relapse and therefore treatment for CTCL continues to be palliative. In this review, we present the current therapeutic concepts for treatment of CTCL including photochemotherapy, interferons, and retinoids and discuss novel treatment options.
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