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Survival, prognostic factors and rates of leukemic transformation in 381 untreated patients with MDS and del(5q): a multicenter study

Myelodysplastic syndromes (MDS) with del(5q) are considered to have a benign course of the disease. In order to address the issue of the propensity of those patients to progress to acute myeloid leukemia (AML), data on 381 untreated patients with MDS and del(5q) characterized by low or intermediate...

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Hauptverfasser: Germing, Ulrich (VerfasserIn) , Lauseker, Michael (VerfasserIn) , Hofmann, Wolf-Karsten (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 13 January 2012
In: Leukemia
Year: 2012, Jahrgang: 26, Heft: 6, Pages: 1286-1292
ISSN:1476-5551
DOI:10.1038/leu.2011.391
Online-Zugang:Verlag, Volltext: http://dx.doi.org/10.1038/leu.2011.391
Verlag, Volltext: https://www.nature.com/articles/leu2011391
Volltext
Verfasserangaben:U. Germing, M. Lauseker, B. Hildebrandt, A. Symeonidis, J. Cermak, P. Fenaux, C. Kelaidi, M. Pfeilstöcker, T. Nösslinger, M. Sekeres, J. Maciejewski, D. Haase, J. Schanz, J. Seymour, M. Kenealy, R. Weide, M. Lübbert, U. Platzbecker, P. Valent, K. Götze, R. Stauder, S. Blum, K.-A. Kreuzer, R. Schlenk, A. Ganser, W.-K. Hofmann, C. Aul, O. Krieger, A. Kündgen, R. Haas, J. Hasford, A. Giagounidis
Beschreibung
Zusammenfassung:Myelodysplastic syndromes (MDS) with del(5q) are considered to have a benign course of the disease. In order to address the issue of the propensity of those patients to progress to acute myeloid leukemia (AML), data on 381 untreated patients with MDS and del(5q) characterized by low or intermediate I International Prognostic Scoring System (IPSS) risk score were collected from nine centers and registries. Median survival of the entire group was 74 months. Transfusion-dependent patients had a median survival of 44 months vs 97 months for transfusion-independent patients (P<0.0001). Transfusion need at diagnosis was the most important patient characteristic for survival. Of the 381 patients, 48 (12.6%) progressed to AML. The cumulative progression rate calculated using the Kaplan-Meier method was 4.9% at 2 years and 17.6% at 5 years. Factors associated with the risk of AML transformation were high-risk World Health Organization adapted Prognostic Scoring System (WPSS) score, marrow blast count >5% and red-cell transfusion dependency at diagnosis. In conclusion, patients with MDS and del(5q) are facing a considerable risk of AML transformation. More detailed cytogenetic and molecular studies may help to identify the patients at risk of progression.
Beschreibung:Gesehen am 05.02.2019
Beschreibung:Online Resource
ISSN:1476-5551
DOI:10.1038/leu.2011.391

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