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.
journal_name
Leukemiajournal_title
Leukemiaauthors
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,doi
10.1038/leu.2011.391subject
Has Abstractpub_date
2012-06-01 00:00:00pages
1286-92issue
6eissn
0887-6924issn
1476-5551pii
leu2011391journal_volume
26pub_type
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