Maligne Lymphome der Haut – Diagnostik und Therapie primär kutaner B-Zell-Lymphome in der Praxis

Zusammenfassung

Primär kutane B-Zell-Lymphome (primary cutaneous B-cell lymphomas: PCBCL) stellen extranodale Non-Hodgkin-Lymphome reifer B-Lymphozyten dar, die die Haut als Zielorgan haben und dort proliferieren. Sie zeigen eine große Bandbreite klinischer und histologischer Erscheinungsformen. Ca. 22 % der kutanen Lymphome (cutaneous lymphomas: CL) entfallen auf kutane B-Zell-Lymphome (cutaneous B-cell lymphomas: CBCL), 73 % auf kutane T-Zell-Lymphome (cutaneous T-cell lymphomas: CTCL) und < 10 % werden seltenen Formen von CL zugeordnet. Die Inzidenz der CL ist weiterhin zunehmend und wird basierend auf den Daten aus den USA auf eine Neuerkrankung pro Jahr und 100 000 Einwohner geschätzt.

Die Diagnose eines PCBCL erfolgt in der Regel anhand klinischer und histologischer bzw. immunhistologischer Untersuchungen. Nicht immer jedoch erlauben diese Untersuchungen eine zweifelsfreie Diagnose. Vor allem frühe Stadien eines PCBCL ähneln sowohl klinisch als auch histologisch oft benignen entzündlichen Hauterkrankungen. Aufgrund der Heterogenität der PCBCL mit unterschiedlicher biologischer Potenz, die von einem indolenten Verlauf bis hin zu einem fatalen Verlauf reicht, ist eine eindeutige Diagnosestellung unabdingbar. Hier spielt insbesondere die Dermatohistologie, in Kombination mit der Immunhistologie und Molekularbiologie, eine entscheidende Rolle.

Abstract

Primary cutaneous B-cell lymphomas (PCBCL) represent extranodal non-Hodgkin’s lymphomas of mature B lymphocytes with the skin as the target organ and the site to proliferate. They show a wide range of clinical and histological features. Approximately 22 % of cutaneous lymphomas (CL) are made up of cutaneous B-cell lymphomas (CBCL) and 73 % of cutaneous T-cell lymphomas (CTCL). The remaining less than 10 % are classified as rare subtypes of CL. The incidence of CL remains increasingly and is estimated at one incidence per year and 100 000 inhabitants. The diagnosis of PCBCL is usually based on clinical, histological and immunohistochemical investigations. But the findings do not always allow an unambiguous diagnosis. Especially early stages of a PCBCL often resemble benign inflammatory skin diseases, both clinically and histologically. Due to the heterogeneity of PCBCL including different biological potencies, ranging from an indolent to a fulminant and fatal course, a distinct diagnosis is essential. Therefore, the correlation of dermatohistology, in combination with immunohistochemistry and molecular biology, plays a crucial role.

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