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BCR-ABL-negative myeloproliferative Neoplasien

BCR-ABL negative myeloproliferative neoplasms

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Zusammenfassung

BCR-ABL-negative myeloproliferative Neoplasien umfassen eine Gruppe von hämatologischen Erkrankungen, die mit der vermehrten Proliferation einer oder mehrer myeloischer Zellreihen einhergehen. Die bekanntesten Vertreter sind Polycythaemia vera, essenzielle Thrombozythämie und Myelofibrose, bei denen in 60–70% der Patienten Mutationen in JAK (Januskinase) oder MPL (myeloproliferatives Leukämie-Virus-Onkogen) gefunden werden, die zu einer konstitutiven Aktivierung der JAK-STAT-Signaltransduktionskaskade führen. Die klinischen und morphologischen Charakteristika des peripheren Blutes und Knochenmarks werden heute standardmäßig durch zytogenetische und molekulargenetische Untersuchungen ergänzt. Präzise Diagnosekriterien und prognostische Marker erlauben in der Regel eine zuverlässige Einschätzung der therapeutischen Optionen. Gegen pathogenetisch bedeutsame molekulare Aberrationen sind zwischenzeitlich effektive Therapien, z. B. JAK-Inhibitoren, entwickelt worden, die möglicherweise in Zukunft als Monotherapie oder in Kombinationen mit und ohne allogener Stammzelltransplantation eingesetzt werden.

Abstract

BCR-ABL negative myeloproliferative neoplasms are a heterogeneous group of disorders characterized by a proliferation of one or more myeloid cell lineages. The most common entities are polycythemia vera, essential thrombocythemia and myelofibrosis in which Janus kinase (JAK) and myeloproliferative leukemia virus oncogene (MPL) mutations are found in 60–70% of patients resulting in constitutive activation of the JAK-STAT signal transduction pathway. The clinical and morphological characteristics in peripheral blood and bone marrow are nowadays regularly complemented by cytogenetic and molecular analyses. Precise diagnostic criteria and prognostic markers allow a reliable evaluation of the therapeutic options. Effective targeted therapies have now been developed against pathogenetic signaling pathways, e.g. JAK inhibitors, which in the future will possibly be used as monotherapy or in combination with or without subsequent allogeneic stem cell transplantation.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: G. Metzgeroth: Vortragshonorare und Reisekostenunterstützung von Novartis. E. Lengfelder: Vortragshonorare und Reisekostenunterstützung von Novartis und Shire. M. Griesshammer: Vortragshonorare und Reisekostenunterstützung von Novartis und Shire. A. Reiter: Vortragshonorare und Reisekostenunterstützung von Novartis, Sanofi und Shire.

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Authors and Affiliations

  1. III. Medizinische Klinik, Universitätsmedizin Mannheim, Theodor-Kutzer Ufer 1-3, 68167, Mannheim, Deutschland

    G. Metzgeroth, E. Lengfelder &  A. Reiter

  2. Hämatologie und Onkologie, Johannes Wesling Klinikum Minden, Minden, Deutschland

    M. Griesshammer

Authors
  1. G. Metzgeroth
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  2. E. Lengfelder
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  3. M. Griesshammer
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  4. A. Reiter
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Correspondence to A. Reiter.

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Metzgeroth, G., Lengfelder, E., Griesshammer, M. et al. BCR-ABL-negative myeloproliferative Neoplasien. Onkologe 18, 1130–1138 (2012). https://doi.org/10.1007/s00761-012-2339-2

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  • DOI: https://doi.org/10.1007/s00761-012-2339-2

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