Zusammenfassung
In diesem Kapitel wird zunächst die Hämatopoese beschrieben: Die Blutbildung im Knochenmark geht von pluripotenten Stammzellen aus, welche an das Stroma der Knochenbälkchen angeheftet sind. Aus diesen entwickeln sich durch Teilung und Differenzierung die Progenitor-, die Vorläufer- und die reifen Zellen der Hämatopoese. Sodann werden die Prinzipien der Diagnostik dargestellt: Nach Anamnese und Untersuchungsbefund wird eine Verdachtsdiagnose gestellt, die durch eine nachfolgende Labordiagnostik bestätigt oder widerlegt werden kann. Sodann werden Ausstrichpräparate von peripherem Blut und Knochenmarkaspiraten mikroskopisch beurteilt. Hierdurch kann die Differenzialdiagnose i. d. R. weiter eingeengt werden und die weitere Diagnostik gezielt erfolgen. Schließlich geht es um die hämatopoetische Stammzelltransplantation (HSZT): Die Anwendung einer intensiven Chemo- und/oder Radiotherapie mit anschließender autologer oder allogener HSZT erlaubt die Behandlung von Patienten, die mit konventioneller Therapie nicht heilbar sind. Die HSZT ist auch die Therapie der Wahl für die meisten Erkrankungen mit Knochenmarkversagen in allen drei Zellreihen. Die genetischen Grundlagen der angeborenen Formen dieser Panzytopenien wie Fanconi-Anämie und Dyskeratosis congenita sind in den letzten Jahren weitestgehend dargestellt worden, während die Ätiologie der erworbenen aplastischen Anämie unklar bleibt.
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Sykora, KW. et al. (2018). Knochenmark. In: Niemeyer, C., Eggert, A. (eds) Pädiatrische Hämatologie und Onkologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43686-8_1
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