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Prognostic impact o...
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.
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Noort, Sanne (author)
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Zimmermann, Martin (author)
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Reinhardt, Dirk (author)
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Cuccuini, Wendy (author)
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Pigazzi, Martina (author)
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Smith, Jenny (author)
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Ries, Rhonda E (author)
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Alonzo, Todd A (author)
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Hirsch, Betsy (author)
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Tomizawa, Daisuke (author)
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Locatelli, Franco (author)
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Gruber, Tanja A (author)
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Raimondi, Susana (author)
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Sonneveld, Edwin (author)
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Cheuk, Daniel K (author)
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Dworzak, Michael (author)
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Stary, Jan (author)
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- Abrahamsson, Jonas, 1954 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics
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Arad-Cohen, Nira (author)
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Czogala, Malgorzata (author)
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De Moerloose, Barbara (author)
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Hasle, Henrik (author)
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Meshinchi, Soheil (author)
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van den Heuvel-Eibrink, Marry (author)
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Zwaan, C Michel (author)
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(creator_code:org_t)
- American Society of Hematology, 2018
- 2018
- English.
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In: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 132:15, s. 1584-1592
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Pediatrik (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Pediatrics (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
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Blood
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- By the author/editor
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Noort, Sanne
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Zimmermann, Mart ...
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Reinhardt, Dirk
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Cuccuini, Wendy
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Pigazzi, Martina
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Smith, Jenny
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show more...
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Ries, Rhonda E
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Alonzo, Todd A
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Hirsch, Betsy
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Tomizawa, Daisuk ...
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Locatelli, Franc ...
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Gruber, Tanja A
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Raimondi, Susana
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Sonneveld, Edwin
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Cheuk, Daniel K
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Dworzak, Michael
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Stary, Jan
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Abrahamsson, Jon ...
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Arad-Cohen, Nira
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Czogala, Malgorz ...
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De Moerloose, Ba ...
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Hasle, Henrik
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Meshinchi, Sohei ...
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van den Heuvel-E ...
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Zwaan, C Michel
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Pediatrics
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cancer and Oncol ...
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
- Articles in the publication
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Blood
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University of Gothenburg