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

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

Leukemia

journal_title

Leukemia

authors

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.2011.391

subject

Has Abstract

pub_date

2012-06-01 00:00:00

pages

1286-92

issue

6

eissn

0887-6924

issn

1476-5551

pii

leu2011391

journal_volume

26

pub_type

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