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Neoplastische Bildungsstörungen der Hämatopoiese mit Ausreifungsverlust

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Zusammenfassung

Die neoplastischen Bildungsstörungen der Hämatopoese mit Ausreifungsverlust umfassen die große und heterogene Gruppe der akuten myeloischen Leukämien. Die gegenwärtige WHO Klassifikation stellt einen Hybrid aus morphologischen und molekularen Definitionsmerkmalen dar. Neben dem Phänotyp und der zugrundeliegenden genetischen Aberration kommen als Klassifikationsprinzip auch weitere Patientenmerkmale hinzu, die Verlauf und Prognose mitbestimmen: Genetische Prädisposition (z.B. Down Syndrom, Fanconi Anämie u.a.), vorbestehende klonale Proliferationen wie MDS oder myelodysplastische/myeloproliferative Neoplasien und eine vorangegangene Chemotherapie. Damit ergibt sich für die Diagnostik ein komplexes Bild aus subtilen morphologischen, immunhistochemischen und vielfältigen, nur partiell mit einem morphologischen Korrelat versehenen, molekulargenetischen Klassifikationsmerkmalen, die in diesem Kapitel in allen Facetten, ausgehend von den zytologischen und histologischen Befunden am Knochenmark, unter Einschluss neudefinierter hämatopoetischer Stammzell- und Vorläuferzellneoplasien dargestellt werden. Eine wichtige Aufgabe ist auch die Remissionsbeurteilung unter Therapie, die ebenfalls behandelt wird.

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Kreipe, H.H. (2019). Neoplastische Bildungsstörungen der Hämatopoiese mit Ausreifungsverlust. In: Müller-Hermelink, H., Kreipe, H. (eds) Pathologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-85184-4_7

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