Lymphomatoide Papulose - ein Schaf im Wolfspelz

Zusammenfassung

Bei der lymphomatoiden Papulose (LyP) handelt es sich um eine CD30-positive T-Zell-lymphoproliferative Erkrankung mit guter Prognose, aber häufig langjährigem, rezidivierendem Verlauf. Bei etwa 10 - 20 % der Patienten mit LyP wird das Auftreten von malignen Lymphomen beobachtet. Aufgrund der Diskrepanz zwischen klinisch benignem Verlauf und malignem histologischen Zellbild wurde die Frage, ob es sich bei der LyP um eine reaktiv-entzündliche Erkrankung oder um ein niedrigmalignes kutanes T-Zell-Lymphom handelt, lange Zeit kontrovers diskutiert. Hinweise auf einen klonalen Ursprung der LyP erbrachten PCR-Untersuchungen der T-Zell-Rezeptorumlagerung in Hautläsionen der LyP und assoziierter maligner Lymphome, die das Vorkommen eines identischen T-Zell-Klons in LyP als auch assoziiertem Lymphom zeigen konnten. Die Vermutung, dass es sich bei der LyP per se um eine monoklonale Erkrankung handelt, ließ sich kürzlich auf Einzelzellebene bestätigen. Dabei wurde gezeigt, dass sich der chronische Verlauf der LyP mit den für diese Erkrankung charakteristischen rezidivierenden, spontan abheilenden Hautläsionen auf die Expansion und Regression eines identischen T-Zell-Klons zurückführen lässt. Dementsprechend wird die LyP sowohl in der EORTC- als auch in der WHO-Klassifikation der Lymphome als niedrigmalignes T-Zell-Lymphom geführt, nimmt jedoch aufgrund des benignen Verlaufs mit einer 10-Jahres-Überlebensrate von 100 % eine Sonderstellung ein.

Abstract

Lymphomatoid papulosis (LyP) represents a cutaneous CD30-positive T-cell lymphoproliferative disorder that takes a clinical benign course, often over a period of several years. From 10 - 20 % of LyP cases are associated with malignant lymphoma. Due to the contrast of benign clinical course and malignant histological picture the nosological position of LyP was a long-standing enigma. On the one hand LyP was assigned to the group of benign reactive processes, on the other hand it was seen as a cutaneous lymphoma. By the finding of the same T-cell receptor rearrangements in the LyP as well as in the associated malignant lymphoma, LyP was identified as a precursor lesion. Recently single-cell analysis of T-cell receptor rearrangements in LyP cases being not associated with malignant lymphoma demonstrated that LyP unequivocally represents a monoclonal T-cell disorder. Moreover it was shown that the prolonged course of LyP with its typical features of waxing and waning is due to the expansion and regression of a single T-cell clone. According to these findings, nowadays LyP is included as a cutaneous low grade malignant lymphoma in both the EORTC- and WHO lymphoma classification. However due to its favourable 10-year survival rate of 100 %, LyP occupies an exceptional position in the spectrum of cutaneous lymphoma.

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