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

Details

Serval ID
serval:BIB_3715D1EBA05D
Type
Article: article from journal or magazin.
Collection
Publications
Title
Survival, prognostic factors and rates of leukemic transformation in 381 untreated patients with MDS and del(5q): a multicenter study.
Journal
Leukemia
Author(s)
Germing U., Lauseker M., Hildebrandt B., Symeonidis A., Cermak J., Fenaux P., Kelaidi C., Pfeilstöcker M., Nösslinger T., Sekeres M., Maciejewski J., Haase D., Schanz J., Seymour J., Kenealy M., Weide R., Lübbert M., Platzbecker U., Valent P., Götze K., Stauder R., Blum S., Kreuzer K.A., Schlenk R., Ganser A., Hofmann W.K., Aul C., Krieger O., Kündgen A., Haas R., Hasford J., Giagounidis A.
ISSN
1476-5551 (Electronic)
ISSN-L
0887-6924
Publication state
Published
Issued date
2012
Volume
26
Number
6
Pages
1286-1292
Language
english
Notes
Publication types: Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
Abstract
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.
Keywords
Adult, Aged, Aged, 80 and over, Cell Transformation, Neoplastic/genetics, Cell Transformation, Neoplastic/pathology, Chromosome Deletion, Chromosomes, Human, Pair 5/genetics, Disease Progression, Female, Humans, Male, Middle Aged, Myelodysplastic Syndromes/diagnosis, Myelodysplastic Syndromes/genetics, Preleukemia/diagnosis, Preleukemia/genetics, Prognosis, Registries, Retrospective Studies, Risk Factors, Survival Rate
Pubmed
Web of science
Open Access
Yes
Create date
05/07/2012 19:06
Last modification date
20/08/2019 14:25
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