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Prognostic impact of t(16;21)(p11;q22) and t(16;21)(q24;q22) in pediatric AML: a retrospective study by the I-BFM Study Group.

Artikel i vetenskaplig tidskrift
Författare Sanne Noort
Martin Zimmermann
Dirk Reinhardt
Wendy Cuccuini
Martina Pigazzi
Jenny Smith
Rhonda E Ries
Todd A Alonzo
Betsy Hirsch
Daisuke Tomizawa
Franco Locatelli
Tanja A Gruber
Susana Raimondi
Edwin Sonneveld
Daniel K Cheuk
Michael Dworzak
Jan Stary
Jonas Abrahamsson
Nira Arad-Cohen
Malgorzata Czogala
Barbara De Moerloose
Henrik Hasle
Soheil Meshinchi
Marry van den Heuvel-Eibrink
C Michel Zwaan
Publicerad i Blood
Volym 132
Nummer/häfte 15
Sidor 1584-1592
ISSN 1528-0020
Publiceringsår 2018
Publicerad vid Institutionen för kliniska vetenskaper, Avdelningen för pediatrik
Sidor 1584-1592
Språk en
Länkar dx.doi.org/10.1182/blood-2018-05-84...
www.ncbi.nlm.nih.gov/entrez/query.f...
Ämneskategorier Hematologi, Cancer och onkologi, Pediatrik

Sammanfattning

To study the prognostic relevance of rare genetic aberrations in acute myeloid leukemia (AML), such as t(16;21), international collaboration is required. Two different types of t(16;21) translocations can be distinguished: t(16;21)(p11;q22), resulting in the FUS-ERG fusion gene; and t(16;21)(q24;q22), resulting in RUNX1-core binding factor (CBFA2T3). We collected data on clinical and biological characteristics of 54 pediatric AML cases with t(16;21) rearrangements from 14 international collaborative study groups participating in the international Berlin-Frankfurt-Münster (I-BFM) AML study group. The AML-BFM cohort diagnosed between 1997 and 2013 was used as a reference cohort. RUNX1-CBFA2T3 (n = 23) had significantly lower median white blood cell count (12.5 × 109/L, P = .03) compared with the reference cohort. FUS-ERG rearranged AML (n = 31) had no predominant French-American-British (FAB) type, whereas 76% of RUNX1-CBFA2T3 had an M1/M2 FAB type (M1, M2), significantly different from the reference cohort (P = .004). Four-year event-free survival (EFS) of patients with FUS-ERG was 7% (standard error [SE] = 5%), significantly lower compared with the reference cohort (51%, SE = 1%, P < .001). Four-year EFS of RUNX1-CBFA2T3 was 77% (SE = 8%, P = .06), significantly higher compared with the reference cohort. Cumulative incidence of relapse was 74% (SE = 8%) in FUS-ERG, 0% (SE = 0%) in RUNX1-CBFA2T3, compared with 32% (SE = 1%) in the reference cohort (P < .001). Multivariate analysis identified both FUS-ERG and RUNX1-CBFA2T3 as independent risk factors with hazard ratios of 1.9 (P < .0001) and 0.3 (P = .025), respectively. These results describe 2 clinically relevant distinct subtypes of pediatric AML. Similarly to other core-binding factor AMLs, patients with RUNX1-CBFA2T3 rearranged AML may benefit from stratification in the standard risk treatment, whereas patients with FUS-ERG rearranged AML should be considered high-risk.

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