Histologische und molekulare Diagnostik als Grundlage für typadaptierte Behandlung und Management kutaner Lymphome[1]

Zusammenfassung

Kutane T-Zell-Lymphome stellen extranodale Non-Hodgkin-Lymphome reifer T-Lymphozyten dar, die die Haut als Zielorgan haben, dort zu Beginn verbleiben und proliferieren. Sie zeigen eine große Bandbreite klinischer und histologischer Erscheinungsformen und Merkmale. Im Gegensatz zu den nodalen Lymphomen sind ca. 80 % der kutanen Lymphome T-Zell-Lymphome (CTCL, engl. cutaneous T cell lymphoma) und ca. 10 – 20 % B-Zell-Lymphome (CBCL, engl. cutaneous B cell lymphoma). Der Rest entspricht seltenen Entitäten wie die CD4+/CD56+-hämatodermischen Lymphome, deren Ursprungszelle Typ 2 dendritischen Zellen entsprechen. Aufgrund der Heterogenität der kutanen Lymphome und ihrer unterschiedlichen biologischen Potenz, die von einem benigne-indolenten bis hin zu einem fatalen Verlauf reicht, ist eine genaue Typisierung des Tumors für das weitere Vorrgehen unabdingbar. Insbesondere die Dermatohistologie, in Kombination mit der Immunhistologie und der Molekularbiologie, spielen eine entscheidende Rolle um die richtige Therapiewahl zu treffen.

Abstract

Cutaneous T-cell lymphomas represent extranodal non-Hodgkin lymphomas consisting of mature lymphocytic populations targeting the skin, persisting and proliferating initially in the skin environment. Cutaneous lymphomas reveal a broad variety of clinical manifestations and histological pictures. In contrast to lymphomas of nodal origin, 80 % of all cutaneous lymphomas (CTCL) derive from T-cell populations, the other 10 – 20 % being of B-cell origin (CBCL). The remaining number of lymphomas correspond to rare entities, such as CD4+/CD56+ hematodermic lymphomas, deriving from type-2 dendritic cells. Based on the heterogeneity of these tumours and their varying biological potency, showing long-term benign or fatal clinical courses, there is a strong need for clarifying the type of the tumour, including its cell of origin. Classical histological examination together with immunochemistry and molecular biological techniques are the most important factors for introducing proper treatment.

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1 Diese Arbeit ist dem 10-jährigen Jubiläum der Berliner Stiftung für Dermatologie (BSD) und der bisher geleisteten Unterstützung an vielen jüngeren Kollegen in ihrer Weiterbildung und Forschung gewidmet.