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Dtsch Med Wochenschr 2006; 131(13): 685-690
DOI: 10.1055/s-2006-933717
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Hämatologie / Onkologie
© Georg Thieme Verlag Stuttgart · New York

Periphere T-Zell-Lymphome - Diagnostik und Therapie

Peripheral T-cell lymphoma: diagnosis and treatmentP. Reimer1 , M. Hentrich2
  • 1Medizinische Klinik und Poliklinik II, Julius-Maximilians-Universität
  • 2Interdisziplinäres Tumorzentrum, Klinikum München-Harlaching
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Publication History

eingereicht: 2.11.2005

akzeptiert: 24.2.2006

Publication Date:
23 March 2006 (online)

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Zusammenfassung

Periphere T/NK-Zell-Lymphome (PTZL) sind eine heterogene Gruppe seltener Erkrankungen, die nur 10 - 15 % aller Non-Hodgkin-Lymphome ausmachen. Im Vergleich zu B-Zell-Lymphomen manifestieren sich PTZL häufiger extranodal und haben eine schlechtere Prognose. Wegen des zumeist günstigen Verlaufs sollten primär kutane T/NK-Zell-Lymphome von den übrigen PTZL getrennt betrachtet werden. Die Stadieneinteilung erfolgt nach der Ann-Arbor-Klassifikation. Der für B-Zell-Lymphome etablierte Internationale Prognostische Index (IPI) ist auch für PTZL relevant.

Eine befriedigende Standardtherapie der PTZL ist bisher nicht definiert. In der Primärtherapie führt eine konventionelle (Anthrazyklin-basierte) Chemotherapie zu Langzeitremissionen in 15 bis 42 % der Patienten. Abgesehen vom prognostisch deutlich günstigeren (ALK-positiven) anaplastischen großzelligen T-Zell-Lymphom bleiben die Ergebnisse somit hinter denen zurück, die bei aggressiven B-Zell-Lymphomen erreicht werden. Die Hochdosistherapie mit autologer Stammzelltransplantation (SZT) ist für rezidivierte und primär refraktäre PTZL eine sinnvolle Therapieoption, weil sie ähnlich effektiv ist wie bei aggressiven B-Zell-Lymphomen. Vielversprechend sind auch bisherige Studienergebnisse zur allogenen SZT nach dosisreduzierter Konditionierung bei Patienten im Rezidiv. Inwieweit die Primärtherapie der PTZL durch Hochdosiskonzepte oder den Einsatz neuerer Substanzen wie z. B. Alemtuzumab verbessert werden kann, ist bislang nicht geklärt. Patienten mit PTZL sollten daher nach Möglichkeit im Rahmen klinischer Studien behandelt werden.

Summary

Peripheral T/NK-cell lymphomas (PTCL) comprise a heterogeneous group of rare diseases accounting for approximately 10-15% of all non-Hodgkin"s lymphomas. Compared to B-cell lymphomas, PTCL more frequently involve extranodal sites and have a worse prognosis. Because of their usually indolent course, primary cutaneous T/NK-cell lymphomas should be distinguished from the other PTCL. Staging of PTCL is done according to the Ann Arbor staging system. The International Prognostic Index, established for aggressive B-cell lymphomas, has also proved relevant for PTCL. Standard therapy for PTCL has not been defined, yet. First line anthracycline-based chemotherapy brings about long-term remissions in 15 to 42% of patients. Apart from (ALK-positive) anaplastic large cell lymphoma it thus gives poorer results than those obtained in patients with aggressive B-cell lymphomas. Data for high-dosage therapy with autologous stem cell transplantation (autoSCT) for relapsing and refractory PTCL are similar to those reported for aggressive B-cell lymphomas. Thus this treatment seems to constitute a sensible salvage strategy. Allogeneic stem cell transplantation following reduced conditioning regimens has also given promising results in patients with relapse. However, the impact of high-dosage strategies and the implementation of newer agents, such as alemtuzumab, in first-line treatment are still uncertain. Hence patients with PTCL should be treated within clinical trials.

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Priv.-Doz. Dr. med. Peter Reimer

Medizinische Klinik und Poliklinik II, Julius-Maximilians-Universität

Klinikstraße 6 - 8

97070 Würzburg

Phone: 0931/20170220

Fax: 0931/20170730

Email: p.reimer@medizin.uni-wuerzburg.de

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