Der Internist

, Volume 54, Issue 6, pp 699–708 | Cite as

Myelodysplastische Syndrome

Schwerpunkt
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Zusammenfassung

Myelodysplastische Syndrome sind klonale Erkrankungen der Blutstammzellen, die in 30–40 % der Fälle in akute myeloische Leukämien übergehen. In anfänglichen Stadien haben die klonalen Zellen zwar einen Wachstumsvorteil und überbevölkern das Knochenmark. Es fehlt ihnen jedoch das Potenzial zur geregelten Ausreifung, sodass trotz eines zellreichen Knochenmarks im Blut Zytopenien unterschiedlicher Ausprägung vorliegen. Die Knochenmarkvorläuferzellen werden apoptotisch. Im weiteren Verlauf akkumulieren jedoch zusätzliche genetische Schäden, die eine verstärkte Proliferation ermöglichen, was den gehäuften Leukämieübergang erklärt. In Frühphasen der Erkrankung stehen daher supportive Maßnahmen oder der Versuch der Stärkung der Resthämatopoese mit Wachstumsfaktoren im Vordergrund. Immunologische und immunmodulatorische Therapieansätze können den malignen Zellklon in manchen Fällen langfristig kontrollieren. Ist die Erkrankung in ein proliferatives Stadium übergetreten, werden zytotoxische Verfahren angewendet. Dabei kommen die intensive Chemotherapie gefolgt von einer allogenen Stammzelltransplantation oder epigenetische Therapiemechanismen zur Proliferationshemmung und Differenzierungsinduktion zum Einsatz.

Schlüsselwörter

Präkanzerosen WHO-Klassifikation Wachstumsfaktoren Lenalidomid Allogene Stammzelltransplantation 

Myelodysplastic syndromes

Abstract

The myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells. The transformation rate to acute myeloid leukemia reaches 30–40 %. In early phases of the disease, the clonal cells have a growth advantage but suffer from premature apoptosis, which explains the paradox of a cellular bone marrow coupled to peripheral blood cytopenias. At later stages, additional genetic aberrations accumulate and lead to proliferation with leukemic transformation. Patients with early MDS benefit from supportive therapy or growth factors. Sometimes, immunological or immunomodulatory treatments can suppress the malignant clone and strengthen normal hematopoiesis for sustained periods. Patients with advanced MDS are usually treated with cytotoxic therapy followed by allogeneic stem cell transplantation or with epigenetic therapy to initiate differentiation and slow down proliferation.

Keywords

Precancerous conditions WHO classification Growth factors Lenalidomide Stem cell transplantation, allogeneic 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Klinik für Onkologie, Hämatologie und PalliativmedizinMarienhospital DüsseldorfDüsseldorfDeutschland

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