Zusammenfassung
Die Aufklärung der molekularen Pathogenese der chronischen myeloischen Leukämie (CML) und die Einführung von Tyrosinkinaseinhibitoren (TKI) haben die Prognose dieser Krankheit erheblich verbessert. Aufgrund der Therapieerfolge der CML nimmt die Prävalenz stetig zu. Blastenkrisen treten mit einer Inzidenz von 1–2% pro Jahr auf; ihre Behandlungsmöglichkeiten sind begrenzt. Zur optimalen Therapie der CML gehört neben der Anwendung moderner Therapien die Nutzung standardisierter molekularzytogenetischer Überwachungsmethoden. Die Anwendung der Zweitgenerations-TKI als Erstlinientherapie erbringt rasche und tiefe molekulare Remissionen mit der besten Chance, die Therapie nach einigen Jahren im Rahmen von Studien beenden zu können. Die CML tritt in allen Altersgruppen auf. Bei einem medianen Alter von 55–60 Jahren sind die Therapien von Kindern und jungen Erwachsenen sowie von älteren Patienten mit Komorbiditäten besondere Herausforderungen. Umfassende Information der Patienten und ihrer Familien über die Art der Erkrankung, die Bedeutung der kontinuierlichen dosisgerechten Medikamenteneinnahme sowie regelmäßige Mitteilung des erreichten zytogenetischen und molekularen Therapieansprechens sind Schlüsselfaktoren für eine optimale Compliance.
Abstract
Elucidation of the pathogenesis of chronic myeloid leukemia (CML) and the introduction of tyrosine kinase inhibitors (TKI) has transformed this disease from being invariably fatal to being the type of leukemia with the best prognosis. However, blast crises occur in 1–2 % of patients per year. Treatment options for blast crises are limited and survival is short. Due to the overall therapeutic success the prevalence of CML is gradually increasing. Optimal management of this disease includes access to modern therapies and standardized surveillance methods for all patients. The first line use of second generation TKIs provides the best chance to achieve fast and deep responses with the potential to eventually discontinue therapy. Standardized laboratory methods are required to provide optimal surveillance according to current recommendations. Comprehensive information on the nature of the disease, the urgent need for day by day drug intake using the appropriate dosages and timely communication of efficacy data are key factors for optimal compliance. All age groups are affected by CML but despite a median age of 55–60 years, particular challenges are the management of the disease in children, young adults and elderly patients with co-morbidities.
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Interessenkonflikt
Der korrespondierende Autor weist auf folgende Beziehungen hin: Andreas Hochhaus: Forschungsunterstützung und Honorare (Novartis, BMS, Ariad, Pfizer, MSD). Thomas Ernst und Janine Ziermann: Keine. Ekkehard Eigendorff: Forschungsunterstützung und Honorare (Novartis). Paul La Rosée: Forschungsunterstützung und Honorare (Novartis, BMS, MSD, Pfizer)
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Hochhaus, A., Ernst, T., Ziermann, J. et al. Chronische myeloische Leukämie. Onkologe 18, 1105–1114 (2012). https://doi.org/10.1007/s00761-012-2352-5
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DOI: https://doi.org/10.1007/s00761-012-2352-5