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Chronische myeloproliferative Erkrankungen

Diagnostik und Therapie

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Zusammenfassung

Die chronischen myeloproliferativen Erkrankungen (CMPE) sind neoplastische Erkrankungen der hämatopoetischen Stammzelle. Sie umfassen die chronische myeloische Leukämie (CML), die Polycythaemia vera, die essentielle Thrombozythämie, die primäre Osteomyelofibrose und Übergangsformen innerhalb dieser Erkrankungen sowie zwischen den CMPE und den Myelodysplasien. Hauptziele der Initialdiagnostik sind die genaue Klassifizierung der Erkrankung, die Erfassung von Risiko- und Prognosefaktoren sowie die Charakterisierung des malignen Klons mittels Zytogenetik und molekulargenetischer Methoden. Herkömmliche Therapeutika für alle CMPE sind Hydroxyurea und Interferon-α. Bei der CML führt die gezielte Hemmung der BCR-ABL-Tyrosinkinase mit Imatinib klinisch zu hohen Ansprechraten. Die bisher einzige kurative Therapie für alle Entitäten ist die allogene Stammzelltransplantation. Bei der CML sollte die Indikation auf der Basis der neueren Risikoscores gestellt werden. Bei den BCR-ABL-negativen CMPE ist sie nur bei ungünstigem Krankheitsverlauf in Erwägung zu ziehen.

Abstract

Chronic myeloproliferative disorders (CMPD) are neoplastic disorders of the hematopoietic stem cell. Four different entities are defined: chronic myeloid leukemia (CML), polycythemia vera, essential thrombocythaemia, and idiopathic myelofibrosis. In addition, overlapping entities within the CMPDs and between CMPDs and myelodysplastic syndrome have been described. Diagnostic measures are performed to classify the subtype exactly and to assess risk factors and prognosis. Cytogenetic and molecular analyses are mandatory for the characterization of the malignant clone. Hydroxyurea and interferon-alpha have proven effective in all CMPE. In CML, specific inhibition of the elevated ABL tyrosine kinase activity with imatinib is associated with high response rates. Allogeneic stem cell transplantation is the only curative treatment option for all entities. In CML, the decision-making analysis should be based on established scores. In BCR-ABL negative CMPDs an allogeneic stem cell transplantation should only be performed in patients with unfavorable prognosis.

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Authors and Affiliations

  1. II. Medizinische Klinik Mannheim, Universität Heidelberg

    E. Lengfelder, U. Berger, A. Reiter, A. Hochhaus & R. Hehlmann

  2. III. Medizinische Klinik Mannheim, Wiesbadener Straße 7–11, 68305, Mannheim

    E. Lengfelder

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  1. E. Lengfelder
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  2. U. Berger
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  3. A. Reiter
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  4. A. Hochhaus
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  5. R. Hehlmann
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Correspondence to E. Lengfelder.

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Lengfelder, E., Berger, U., Reiter, A. et al. Chronische myeloproliferative Erkrankungen. Internist 44, 1011–1030 (2003). https://doi.org/10.1007/s00108-003-0974-9

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  • DOI: https://doi.org/10.1007/s00108-003-0974-9

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