Zusammenfassung
Myeloproliferative Erkrankungen beruhen auf einer Veränderung der Knochenmarkstammzelle mit konsekutiver Expansion der klonalen Hämatopoese in allen Zellreihen. Abgesehen von Patienten mit chronisch-myeloischer Leukämie (CML), die durch den Übergang in eine rasch tödlich verlaufende Blastenkrise bedroht sind, leiden Patienten mit chronischmyeloproliferativen Erkrankungen (cMPE) neben Symptomen der extramedullären Blutbildung und Organomegalie vorwiegend unter Blutungen, Thrombosen und Mikrozirkulationsstörungen. Komplikationshäufigkeit und Symptomatik variieren bei den einzelnen Erkrankungen erheblich, so daß die Polycythaemia vera überwiegend durch Thromboseneigung, die essentielle Thrombozythämie durch zerebrale und periphere Durchblutungsstörungen und die Osteomyelofibrose durch Blutungen gekennzeichnet ist. Durch Einbeziehung der Megakaryozytopoese in den Krankheitsprozeß werden morphologisch und funktionell abnorme Blutplättchen gebildet, die primär als Auslöser dieser Komplikationen betrachtet werden. Dabei ist eine Vielzahl von Störungen beschrieben, deren Beitrag zur klinischen Symptomatik jedoch nicht belegt ist. Strittig ist auch, ob den Komplikationen eine megakaryozytäre Bildungsstörung oder eine Thrombozytenaktivierung in der Zirkulation zugrunde liegt. Neben thrombozytären Anomalien darf aber die Proliferation der übrigen Zellreihen und ihre Interaktion mit Endothelzellen als bedeutender pathogenetischer Faktor für Hämostasestörungen nicht außer acht gelassen werden. Als Prognosefaktoren für vaskuläre Komplikationen konnten bisher allerdings nur Art und Stadium der Erkrankung, vorausgegangene Ereignisse und Hämatokrit > 45% gesichert werden. Dagegen haben Thrombozytenzahl und Alter bei Erstdiagnose sowie Thrombozytenfunktionsuntersuchungen in vitro bisher keine sichere prädiktive Bedeutung für Blutungen oder Thrombosen. Zur Therapie und Prophylaxe thrombohämorrhagischer Komplikationen stehen Zytoreduktion und Plättchenfunktionshemmung zur Verfügung. Die Normalisierung der Zellzahlen sollte heute überwiegend mittels Aderlaß, Hydroxyharnstoff und Interferon-α erfolgen und kann nicht nur die Hämostasekomplikationen, sondern auch die Gesamtsymptomatik günstig beeinflussen. Mikrozirkulationsstörungen sprechen besonders gut auf Acetylsalicylsäure an, wobei durch Verwendung niedriger Dosierungen die sonst häufigen Blutungskomplikationen reduziert werden konnten. Bei optimaler therapeutischer Einstellung ist die Lebensqualität der Patienten mit cMPE heute kaum eingeschränkt und die Überlebenszeit mit Ausnahme der CML und fortgeschrittener Osteomyelofibrose nicht verkürzt.
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Wehmeier, A. (1996). Hämostasestörungen bei myeloproliferativen Erkrankungen. In: Spanuth, E. (eds) Malignome und Hämostase. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-79744-6_3
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