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Der Klinikarzt 2020; 49(11): 490-501
DOI: 10.1055/a-1301-4102
Schwerpunkt

Myelodysplastische Syndrome

Diagnostik, Differenzialdiagnostik, Prognose, Therapie und allogene Transplantation
Thomas Schroeder∗
1   Klinik für Hämatologie, Onkologie und Klinische Immunologie, Heinrich-Heine-Universität, Düsseldorf
,
Christina Rautenberg
1   Klinik für Hämatologie, Onkologie und Klinische Immunologie, Heinrich-Heine-Universität, Düsseldorf
,
Ulrich Germing
1   Klinik für Hämatologie, Onkologie und Klinische Immunologie, Heinrich-Heine-Universität, Düsseldorf
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ZUSAMMENFASSUNG

Myelodysplastische Syndrome (MDS) umfassen klonale Stammzellerkrankungen, die mit Dysplasiezeichen, zytogenetischen und molekulargenetischen Veränderungen sowie zum Teil mit einem erhöhten Blastenanteil einhergehen. Die Patienten weisen vor allem klinische Zeichen der hämatopoietischen Insuffizienz, insbesondere Anämiesymptome, auf. Eine exakte und umfassende Diagnostik ist zur Diagnosestellung unerlässlich. Die Prognose wird durch das Progressionsrisiko in eine akute myeloische Leukämie, sowie von zytopeniebedingten Komplikationen und patienteneigenen Faktoren wie Alter und Komorbiditäten beeinflusst. Scores wie das revidierte International Prognostic Scoring System (IPSS-R) dienen der Prognoseabschätzung und somit der risikoadaptierten Therapieplanung. Niedrigrisiko-Patienten werden mit supportiven Maßnahmen wie Transfusionen, Erythropoietin, Lenalidomid bei del(5q) und Eisenchelation behandelt. Die Therapie der Hochrisikopatienten zielt auf die Verlängerung der Lebenserwartung ab und beinhaltet, wenn immer möglich, die allogene Stammzelltransplantation in kurativer Intention und die Gabe von 5-Azacitidine in palliativer Intention. Für die Mehrheit der Patienten steht aber keine zugelassene Therapie zur Verfügung, weshalb diese Patienten in klinischen Studien behandelt werden sollten. Luspatercept wird vermutlich in Kürze eine Zulassung bekommen.



Publication History

Article published online:
25 November 2020

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