Myelodysplastische Syndrome (MDS) View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2009-06

AUTHORS

Thomas Luft, Peter Dreger

ABSTRACT

Myelodysplastische Syndrome (MDS) sind eine heterogene Gruppe klonaler Stammzellerkrankungen, gekennzeichnet durch eine ineffektive, dysplastische Hämatopoese, periphere Zytopenien und eine variable Progressionsrate zur akuten myeloischen Leukämie (AML). Allerdings sind klonale Stammzellen bisher nur bei etwa 50% der Patienten mit MDS zytogenetisch nachweisbar. Daher müssen im Einzelfall schwierige differentialdiagnostische Entscheidungen anhand morphologischer Kriterien gestellt werden, die mit einer erheblichen subjektiven Variabilität belastet sind. Auch die neue Klassifikation der Weltgesundheitsorganisation (WHO) aus dem Jahr 2008 konnte diesem Dilemma nicht abhelfen. Die prinzipielle Heilbarkeit der Erkrankung durch eine allogene Stammzelltransplantation (SZT) erhöht aufgrund der erheblichen transplantationsassoziierten Mortalität und Morbidität die Verantwortung des diagnosestellenden Arztes. Die Verfügbarkeit palliativer, spezifischer Therapien wie 5-Azacytidin (Vidaza®), 5-Aza-2’-Deoxycytidin (Decitabin®) oder Lenalidomid (Revlimid®) ist weltweit unterschiedlich geregelt. Dabei basieren die Indikationen zu 5-Azacytidin wie auch zur SZT auf dem International Prognostic Scoring System (IPSS), bei dem minimale Unterschiede in den Blastenzählergebnissen zu enormen klinischen Konsequenzen führen. Die wichtigste Aufgabe der nächsten Jahre ist daher die Suche nach molekularen prognostischen Markern, die ein objektivierbares Staging erlauben und die Sicherheit der Therapieindikationen erhöhen. More... »

PAGES

59-66

Journal

TITLE

Onkopipeline

ISSUE

2

VOLUME

2

Author Affiliations

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s15035-009-0154-z

DOI

http://dx.doi.org/10.1007/s15035-009-0154-z

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1001555928


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