Zurück zur Übersicht
| Blood. 2015 Jul 27. pii: blood-2015-02-629204. doi: 10.1182/blood-2015-02-629204 |
| Heterogeneous cytogenetic subgroups and outcomes in childhood acute megakaryoblastic leukemia: a retrospective international study. |
| Inaba H1, Zhou Y2, Abla O3, Adachi S4, Auvrignon A5, Beverloo HB6, de Bont E7, Chang TT8, Creutzig U9, Dworzak M10, Elitzur S11, Fynn A12, Forestier E13, Hasle H14, Liang DC15, Lee V16, Locatelli F17, Masetti R18, De Moerloose B19, Reinhardt D20, Rodriguez L21, Van Roy N22, Shen S23, Taga T24, Tomizawa D25, Yeoh AE26, Zimmermann M27, Raimondi SC28 |
 Author information
|
|
Abstract Comprehensive clinical studies of patients with acute megakaryoblastic leukemia (AMKL) are lacking. We performed an international retrospective study on 490 patients (age ≤18 years) with non-Down syndrome de novo AMKL diagnosed from 1989 to 2009. Patients with AMKL (median age 1.53 years) comprised 7.8% of pediatric AML. Five-year event-free (EFS) and overall survival (OS) were 43.7%±2.7% and 49.0%±2.7%, respectively. Patients diagnosed in 2000-2009 were treated with higher doses of cytarabine and had better EFS (P=0.037) and OS (P=0.003) than those diagnosed in 1989-1999, but outcomes of patients diagnosed in 2000-2004 and 2005-2009 were similar. Transplantation in first remission did not improve survival. Cytogenetic data were available for 372 (75.9%) patients: hypodiploid (n=18, 4.8%), normal karyotype (n=49, 13.2%), pseudodiploid (n=119, 32.0%), 47-50 chromosomes (n=142, 38.2%), and >50 chromosomes (n=44, 11.8%). Chromosome gain occurred in 195 of 372 (52.4%) patients: +21 (n=106, 28.5%), +19 (n=93, 25.0%), +8 (n=77, 20.7%). Losses occurred in 65 patients (17.5%): -7 (n=13, 3.5%). Common structural chromosomal aberrations were t(1;22)(p13;q13) (n=51, 13.7%) and 11q23 rearrangements (n=38, 10.2%); t(9;11)(p22;q23) occurred in 21 patients. On the basis of frequency and analysis for EFS and OS, AMKL can be classified to 3 risk groups: good risk: 7p abnormalities; poor risk: normal karyotypes, -7, 9p abnormalities including t(9;11)(p22;q23)/MLL-MLLT3, -13/13q-, and -15; and intermediate risk: others including t(1;22)(p13;q13)/OTT-MAL and 11q23/MLL except t(9;11). Risk-based innovative therapy is needed to improve patient outcomes. |
|
Copyright © 2015 American Society of Hematology. |
|
Publikations ID: 26215111 Quelle: öffnen |
Author information
Schnellinfo
-- Cancer Care
-- Kliniken und Partner
-- CCC Cancer School
-- Young CCC
-- CCC Tumorboards
-- CCC Forschungscluster
-- CCC Units
-- CCC Platforms
-- SOPs / Leitlinien
-- Kontakt
Featured
