Periphere T-Zell-Lymphome: Aktuelle und zukünftige Therapien

Periphere T-Zell-Lymphome (PTCL) sind mit einer Inzidenz von ca. einer Neuerkrankung pro 100000 Personen im Jahr selten. Unter Erstlinientherapie sind refraktäre und rezidivierte Verläufe häufig, die Prognose in dieser Situation ist schlecht. In der klinischen Praxis ist daher die Rezidivtherapie von großer Bedeutung. Dieser Artikel gibt einen Überblick der aktuellen Therapien und zeigt, welche zukünftig eine wichtige Rolle spielen könnten.

Kernaussagen

• Die Erstlinientherapie für die meisten T-Zell-Lymphome besteht weiterhin aus dem Standard-Chemotherapieregime CHOP, einer Kombination der klassischen Zytostatika Cyclophosphamid, Doxorubicin, Vincristin und Prednison.

• Eine Ausnahme bildet die Entität der anaplastisch großzelligen T-Zell-Lymphome, für die seit 2020 Brentuximab-Vedotin in Kombination mit Cyclophosphamid, Hydroxydaunorubicin und Prednison in der Primärtherapie zugelassen ist.

• Refraktäre und rezidivierte Verläufe sind in der Erstlinientherapie häufig, die Prognose ist in dieser Situation schlecht.

• Die allogene Stammzelltransplantation stellt für diese Patient*innen aktuell die einzige realistische Chance auf langfristige Heilung dar, jedoch mit relevanter therapieassoziierter Mortalität und Morbidität.

• Daher sind Rezidivtherapien in der klinischen Praxis von großer Bedeutung. Unter anderem epigenetische Modulatoren spielen hierbei eine wichtige Rolle, für die Subentitäten von PTCL eine hohe Sensibilität aufweisen.

• Für Patient*innen, die sich nicht für eine allogene SZT eignen, sind zielgerichtete Therapieansätze im Off-Label-Use oder im Rahmen von Studien von großer Relevanz.

• Zukünftig könnten auch CAR-T-Zellen im Kontext von T-Zell-Lymphomen eine wichtige Rolle spielen, obwohl sich diese Therapieoption derzeit noch am Anfang ihrer Entwicklung befindet und in Studien geprüft wird.

Schlüsselwörter

PTCL - Brentuximab-Vedotin - Epigenetische Modulatoren - CAR-T-Zellen

Publication History

Article published online:
02 December 2024

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