Zusammenfassung
Das Mantelzelllymphom ist durch eine charakteristische Cyclin-D1-Überexpression und einen relativ rasch rezidivierenden Verlauf gekennzeichnet. Histologie und Klinik können jedoch stark variieren, sodass zur individuellen Risikoabschätzung histologische, klinische und biologische Faktoren herangezogen werden. Für die große Mehrheit der Fälle sollte jedoch nach Diagnosestellung rasch eine entsprechende Therapie eingeleitet werden. Bei jüngeren Patienten wiesen mehrere Studien den Vorteil dosisintensivierter Ansätze mit einer autologen Stammzelltransplantation nach, eine aktuelle randomisierte Phase-III-Studie belegt den zusätzlichen Vorteil eines cytarabinhaltigen Regimes. Bei älteren Patienten erzielt eine kombinierte Immunchemotherapie initial hohe Ansprechraten, zur Erzielung lang anhaltender Remissionen sind jedoch weitere Konsolidierungsstrategien notwendig. In einer kürzlich präsentierten Studie des Europäischen MCL Netzwerks führte eine Rituximaberhaltung zu einer mittleren Remissionsdauer von ca. 6 Jahren. Aktuelle Studienkonzepte prüfen Kombinationen mit molekular gezielten Substanzen (Proteasominhibitoren, IMIDs und mTOR-Inhibitoren).
Abstract
Mantle cell lymphoma is characterized by a specific overexpression of cyclin D1 and a rapid relapse pattern. The histological results and clinical course may differ significantly so that histological, clinical and biological factors should be considered for the individual risk profile. However, treatment should be immediately initiated in the vast majority of cases. In younger patients, the advantage of dose intensification with autologous stem cell transplantation has been proven in numerous trials and a recent randomized phase III study confirmed the additional benefit of a cytarabine-containing regimen. In elderly patients combined immunochemotherapy achieves an initial high response rate but additional consolidation is crucial to achieve long lasting remission. In a recent study of the European MCL network a rituximab maintenance achieved a median duration of remission of almost 6 years. Current study concepts investigate the addition of molecular targeted approaches (proteasome inhibitors, IMIDS and mTOR inhibitors).
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Interessenkonflikt
Der korrespondierende Autor weist auf folgende Beziehungen hin:
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Wissenschaftliche Advisory Boards: Celgene, Mundipharma, Pfizer, Roche.
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Finanzielle Unterstützung von unabhängigen klinischen Studien (IITs): Celgene, Janssen, Mundipharma, Roche.
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Finanzielle Unterstützung von präklinischen Studien: GSK, Roche.
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Rednerhonorare: Celgene, Janssen, Mundipharma, Pfizer, Roche.
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Dreyling, M. Therapie des Mantelzelllymphoms. Onkologe 17, 806–813 (2011). https://doi.org/10.1007/s00761-011-2129-2
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DOI: https://doi.org/10.1007/s00761-011-2129-2
Schlüsselwörter
- Mantelzelllymphom
- Cyclin-D1-Überexpression
- Autologe Stammzelltransplantation
- Rituximab
- Molekulare Strategien