Der klinische Verlauf der chronischen myeloproliferativen Erkrankungen ist sehr variabel. Bei der essentiellen Thrombozythämie und Polycythaemia vera stehen vaskuläre Komplikationen im Vordergrund, während die primäre Myelofibrose meist durch Zytopenie und ein hohes Risiko der Transformation in eine sekundäre akute Leukämie gekennzeichnet ist. Die Entdeckung der Mutation V617F im Gen der Januskinase 2 (JAK2) im Jahr 2005 hat die Diagnostik der chronischen myeloproliferativen Erkrankungen in vielen Fällen vereinfacht und zur Revision der WHO-Diagnosekriterien geführt. Der Beitrag bietet einen Überblick über den aktuellen Stand bei Diagnostik und Therapie von essentieller Thrombozythämie, Polycythaemia vera und primärer Myelofibrose.
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Stegelmann, F., Kvasnicka, H.M. & Griesshammer, M. Chronische myeloproliferative Erkrankungen. best practice onkologie 3, 24–32 (2008). https://doi.org/10.1007/s11654-008-0050-y
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DOI: https://doi.org/10.1007/s11654-008-0050-y