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DOI: 10.1055/s-0032-1305003
Chronische lymphatische Leukämie
Chronic lymphocytic leukemiaPublication History
Publication Date:
22 May 2012 (online)
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Diagnostik: Die Diagnose wird aus einer durchflusszytometrischen Analyse des peripheren Blutes gestellt, in der Regel besteht keine Notwendigkeit einer diagnostischen Knochenmarkpunktion. Vor Therapieeinleitung (prinzipiell definiert durch fortgeschrittenes Erkrankungsstadium Binet C) muss ein TP53-Defekt mittels Interphase-FISH (17p-) bzw. Mutationsanalyse (p53 Mutation) ausgeschlossen werden.
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Therapie: Art und Umfang der Therapie werden durch Fitness, nicht kalendarisches Alter, des Patienten definiert. Für fitte Patienten wurde das FCR-Schema als neuer Erstlinienstandard etabliert. Bei weniger fitten Patienten sollte Chlorambucil oder Bendamustin erwogen werden. Bei TP53-Defekt wird eine Alemtuzumab-haltige Therapie, evtl. mit hochdosierten Steroiden, empfohlen. Bei jungen und fitten Patienten ist allein die allogene Transplantation kurativ.
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Klinischer Ausblick: Chemotherapiefreie Therapieoptionen auf der Basis von Lenalidomid mit oder ohne Antikörper sind in Entwicklung. Erste vielversprechende Ansätze bieten Tyrosinkinase-Inhibitoren, die verschiedene Signalwege des B-Zellrezeptors hemmen (u. a. CAL-101, PCI32765) und bereits in der Monotherapie Ansprechraten bis zu 70 % erzielen. Weitere Therapiefortschritte für Patienten mit CLL sind allein durch konsequenten Einschluss in klinische Studien zu erzielen.
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Literatur
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