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Chronische myeloische Leukämie

Diagnostik, Therapie und Zukunftsstrategie

Chronic myeloid leukemia

Diagnostics, therapy and future strategy

Der Internist volume 52, pages 283–296 (2011)Cite this article

Zusammenfassung

Das Überleben der Patienten mit chronischer myeloischer Leukämie (CML) hat sich mit der Einführung des BCR-ABL-spezifischen Tyrosinkinaseinhibitors Imatinib dramatisch verbessert. In der Regel erreichen Patienten unter Imatinib anhaltende komplette zytogenetische und molekulare Remissionen. Nur eine Minderheit der Patienten ist primär refraktär gegenüber Imatinib oder verliert eine zuvor erreichte Remission unter Imatinib. Diese Gruppe hat dann eine schlechtere Prognose. Diese Übersicht gibt eine übersichtliche Darstellung der erforderlichen Diagnostik bei Erstdiagnose einer CML und der Beurteilung des Ansprechens im Verlauf. Die hierfür entwickelten Leitlinien des Europäischen Leukämienetzes zu Art und Frequenz von Verlaufskontrollen sowie die diagnostischen Kriterien für Imatinibresistenz oder suboptimales Ansprechen werden erörtert. Die Indikation zur Durchführung einer allogenen Stammzelltransplantation und der Einsatz von Zweitgenerations-BCR-ABL-Inhibitoren als therapeutische Alternativen im Falle von Imatinibversagen werden stadienspezifisch diskutiert. Zuletzt wird ein Ausblick auf Therapieziele und -formen einer zukünftigen Erstlinientherapie der CML gegeben.

Abstract

Survival of patients with chronic myeloid leukemia (CML) has dramatically improved with the introduction of the BCR-ABL-specific tyrosine kinase inhibitor imatinib. As a rule patients on therapy with imatinib achieve permanent complete cytogenetic and molecular remission. Patients who are primarily refractive to imatinib or lose remission achieved using imatinib are in the minority. This group has a poor prognosis. This article gives a transparent review of the diagnostics necessary when CML is primarily diagnosed and for assessment of the response during the course of the therapy. The guidelines developed for this procedure by the European leukemia network on the type and frequency of surveillance controls as well as the diagnostic criteria for imatinib resistance or suboptimal response will be presented. The indications for allogenic stem cell transplantation and the administration of second generation BCR-ABL inhibitors will be discussed as therapeutic alternatives in cases of imatinib failure in a stage-specific manner. Finally a view on therapy targets and forms of future first-line therapy of CML will be given.

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Danksagung

Diese Arbeit wurde durch die Deutsche José Carreras Leukämie-Stiftung e.V., DJCLS 06/01v (A.B.), die Deutsche Forschungsgemeinschaft, KFO210 (BU 1321/3–1, NE 310/14–1) (A.B. und A.N.) und eine Forschungsförderung des Universitätsklinikums Gießen und Marburg (A.B.) unterstützt.

Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin:

Forschungsunterstützung Novartis, Referententätigkeit BMS.

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Affiliations

  1. Klinik für Hämatologie, Onkologie und Immunologie, Philipps Universität Marburg, Universitätsklinikum Giessen und Marburg, Baldingerstraße, 35043, Marburg, Deutschland

    A. Burchert &  A. Neubauer

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  1. A. Burchert
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  2. A. Neubauer
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Correspondence to A. Burchert or A. Neubauer.

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Burchert, A., Neubauer, A. Chronische myeloische Leukämie. Internist 52, 283–296 (2011). https://doi.org/10.1007/s00108-010-2791-2

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  • DOI: https://doi.org/10.1007/s00108-010-2791-2

Schlüsselwörter

  • Chronische myeolische Leukämie
  • Tyrosinkinaseinhibitoren
  • Imatinib
  • Kinaseinhibitorresistenz
  • Verlaufsdiagnostik

Keywords

  • Chronic myeloid leukemia
  • Tyrosine kinase inhibitors
  • Imatinib
  • Kinase inhibitor resistance
  • Course diagnostics