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Hypoplastische Anämien

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Pädiatrische Hämatologie und Onkologie

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Zusammenfassung

Aplastische Anämie (chronische Erythroblastophthise) und Cortison

Von C. Gasser

»Echte aplastische Anämie im engeren Sinne oder chronische Erythroblastophthise ist ein sehr seltenes Ereignis (Fälle von Heilmeyer, Moeschlin und Rohr, Begemann), bei dem es im Knochenmark zu einem vollstä ndigen Schwund der Erythroblasten über Monate und Jahre kommt, und zwar ohne wesentliche Beeinträchtigung der Leuko- und Thrombopoese. In der pädiatrischen Literatur wird unter »aplastischer Anämie« meist eine kongenital auftretende, hypoplastische Anämie mit wohl starker Verminderung, aber nicht vollstä ndigem Ausfall der Erythroblasten im Knochenmark und der Retikulozyten im Blut verstanden. Diese Er krankungsform bezeichnet Glanzmann wohl mit Recht als »essentielle Erythroblastopenie mit Anämie vom Typus Josephs-Diamond-Blackfan«. Sie stellt das Gegenstück der von uns beobachteten akuten Form der Erkrankung (»akute Erythroblastopenie«) dar. ...

In dieser Veröffentlichung aus dem Jahr 1951 (Schweizerische Medizinische Wochenzeitschrift 50:1241–1242) beschreibt Gasser erstmalig das Ansprechen eines Kindes mit Diamond-Blackfan-Anämie auf Kortison. Der Begriff aplastische Anämie ist heute den Panzytopenien vorbehalten, die Diamond-Blackfan-Anämie wie auch » ihr Gegenstück«, die akute transitorische Erythroblastopenie des Kindesalters, werden hingegen als hypoplastische Anämien eingeordnet.

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Authors and Affiliations

  1. Zentrum für Kinderheilkunde und Jugendmedizin Klinik IV: Pä diatrische Hämatologie und Onkologie, Universitätsklinikum Freiburg, Mathildenstr. 1, 79106, Freiburg

    Prof. Dr. Charlotte Niemeyer & Dr. Jörg Meerpohl

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  1. Prof. Dr. Charlotte Niemeyer
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  2. Dr. Jörg Meerpohl
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Editors and Affiliations

  1. Forschungsinstitut für krebskranke Kinder, St. Anna Kinderspital, Kinderspitalgasse 6, 1090, Wien, Österreich

    Helmut Gadner

  2. Klinik für Allgemeine Pädiatrie und Knochenmarktransplantation, Charité Campus Virchow-Klinikum Otto-Heubner-Centrum für Kinder- und Jugendmedizin, Augustenburger Platz 1, 13353, Berlin

    Gerhard Gaedicke

  3. Zentrum für Kinderheilkunde und Jugendmedizin, Universitätsklinikum Freiburg, Mathildenstr. 1, 79106, Freiburg

    Charlotte Niemeyer

  4. Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Münster, Albert-Schweitzer-Str. 33, 48129, Münster

    Jörg Ritter

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Niemeyer, C., Meerpohl, J. (2006). Hypoplastische Anämien. In: Gadner, H., Gaedicke, G., Niemeyer, C., Ritter, J. (eds) Pädiatrische Hämatologie und Onkologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-29036-2_6

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  • DOI: https://doi.org/10.1007/3-540-29036-2_6

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