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Diagnostic algorithms, monitoring, prognostication, and therapy in chronic myeloid leukemia (CML): a proposal of the Austrian CML platform

Diagnostische Algorithmen, Prognose und Therapie in der Chronisch Myeloischen Leukämie (CML): Ein Proposal der Österreichischen CML-Plattform

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Zusammenfassung

Die chronisch myeloische Leukämie ist eine Stammzellerkrankung, welche durch das BCR/ABL Onkoprotein gekennzeichnet ist. Der ABL Kinase-Inhibitor Imatinib ist in den meisten Patienten wirksam und gilt als Standard in der Erstlinientherapie. Allerdings kommt es in einem Teil der Patienten zur Resistenzentwicklung. Für diese Patienten stehen neue Multikinase-Inhibitoren wie Dasatinib oder Nilotinib sowie die Stammzelltransplantation als prinzipielle Therapie-Optionen zur Verfügung. Die Entscheidung bezüglich der Therapie und Wahl der Medikamente richtet sich nach dem Vorliegen und der Art der BCR/ABL Mutationen, der Phase der Erkrankung, pharmakologischen Parametern, sowie patienten-bezogenen Faktoren wie Alter und Komorbidität. Der vorliegende Artikel fasst die derzeit als Standard geltenden Strategien zur Diagnostik und Therapie der frisch-diagnostizierten und der imatinib-resistenten CML zusammen, und präsentiert entsprechende Algorithmen, welche in der CML Plattform der Österreichischen Gesellschaft für Hämatologie und Onkologie (ÖGHO) in den Jahren 2007 und 2008 diskutiert und erstellt wurden. Die resultierenden Empfehlungen sollten in der Patientenbetreuung und Therapie, in der Auswahl der optimalen diagnostischen Tests und therapeutischen Verfahren in der klinischen Praxis, sowie in der Planung von klinischen Studien einen hilfreichen Beitrag leisten.

Summary

Chronic myeloid leukemia (CML) is a stem cell disease characterized by the BCR/ABL oncoprotein. The ABL kinase inhibitor imatinib is effective in most patients and considered standard first-line therapy. However, not all patients show a long-lasting response to this drug. In fact, resistance against imatinib has been described and is an emerging clinical problem in CML. For these patients, novel multi-kinase inhibitors such as nilotinib or dasatinib as well as stem cell transplantation, represent alternative treatment options. The decision concerning second-line therapies and selection of drugs is usually based on the presence and type of BCR/ABL mutations, the phase of disease, other disease-related factors as well as patient-related factors including age, co-morbidity, and pharmacologic determinants. The current article provides an overview on diagnostic and therapeutic strategies for patients with treatment-naïve and imatinib-resistant CML, together with proposed algorithms that were discussed and approved by members of the CML platform of the Austrian Society for Hematology and Oncology (ÖGHO) in 2007 and 2008. The resulting recommendations should assist in diagnosis and prognostication in CML, follow-up and disease-monitoring, patient selection for interventional therapies, and in the preparation and conduct of clinical trials.

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Authors and Affiliations

  1. Department of Internal Medicine I, Division of Haematology & Hemostaseology, Medical University of Vienna, Austria

    Peter Valent, Christian Sillaber & Hermine Agis

  2. Ludwig Boltzmann Cluster Oncology, Vienna, Austria

    Peter Valent

  3. Labdia Labordiagnostik, Vienna, Austria

    Thomas Lion

  4. St.Anna Kinderkrebsforschung, Vienna, Austria

    Thomas Lion

  5. Department of Hematology and Oncology, Medical University Innsbruck, Austria

    Dominik Wolf & Günther Gastl

  6. Krankenhaus der Barmherzigen Schwestern, Linz, Austria

    Andreas Petzer

  7. Department of Internal Medicine, State Hospital, Feldkirch, Austria

    Alois Lang

  8. Department of Internal Medicine I, Bone Marrow Transplantation Unit, Medical University of Vienna, Austria

    Peter Kalhs

  9. First Medical Department, Hospital Klagenfurt, Austria

    Dietmar Geissler

  10. Laboratory for Immunological and Molecular Cancer Research, IIIrd Medical Department, Paracelsus Medical University Salzburg, Austria

    Richard Greil

  11. Department of Clinical Hematology, Clinic for Internal Medicine, Medical University of Graz, Austria

    Werner Linkesch

  12. Fourth Department of Internal Medicine, Hospital Wels-Grieskirchen, Austria

    Sonja Burgstaller & Josef Thaler

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  1. Peter Valent
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  2. Thomas Lion
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Correspondence to Peter Valent.

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Valent, P., Lion, T., Wolf, D. et al. Diagnostic algorithms, monitoring, prognostication, and therapy in chronic myeloid leukemia (CML): a proposal of the Austrian CML platform. Wien Klin Wochenschr 120, 697–709 (2008). https://doi.org/10.1007/s00508-008-1100-8

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