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Statements

Subject Item
n2:RIV%2F61989592%3A15110%2F13%3A33144176%21RIV14-MSM-15110___
rdf:type
n5:Vysledek skos:Concept
dcterms:description
In pediatric acute myeloid leukemia (AML), cytogenetic abnormalities are strong indicators of prognosis. Some recurrent cytogenetic abnormalities, such as t(8;16)(p11;p13), are so rare that collaborative studies are required to define their prognostic impact. We collected the clinical characteristics, morphology, and immunophenotypes of 62 pediatric AML patients with t(8;16)(p11;p13) from 18 countries participating in the International Berlin-Frankfurt-Münster (I-BFM) AML study group. We used the AML-BFM cohort diagnosed from 1995-2005 (n = 543) as a reference cohort. Median age of the pediatric t(8;16)(p11;p13) AML patients was significantly lower (1.2 years). The majority (97%) had M4-M5 French-American-British type, significantly different from the reference cohort. Erythrophagocytosis (70%), leukemia cutis (58%), and disseminated intravascular coagulation (39%) occurred frequently. Strikingly, spontaneous remissions occurred in 7 neonates with t(8;16)(p11;p13), of whom 3 remain in continuous remission. The 5-year overall survival of patients diagnosed after 1993 was 59%, similar to the reference cohort (P = .14). Gene expression profiles of t(8;16)(p11;p13) pediatric AML cases clustered close to, but distinct from, MLL-rearranged AML. Highly expressed genes included HOXA11, HOXA10, RET, PERP, and GGA2. In conclusion, pediatric t(8;16)(p11;p13) AML is a rare entity defined by a unique gene expression signature and distinct clinical features in whom spontaneous remissions occur in a subset of neonatal cases. In pediatric acute myeloid leukemia (AML), cytogenetic abnormalities are strong indicators of prognosis. Some recurrent cytogenetic abnormalities, such as t(8;16)(p11;p13), are so rare that collaborative studies are required to define their prognostic impact. We collected the clinical characteristics, morphology, and immunophenotypes of 62 pediatric AML patients with t(8;16)(p11;p13) from 18 countries participating in the International Berlin-Frankfurt-Münster (I-BFM) AML study group. We used the AML-BFM cohort diagnosed from 1995-2005 (n = 543) as a reference cohort. Median age of the pediatric t(8;16)(p11;p13) AML patients was significantly lower (1.2 years). The majority (97%) had M4-M5 French-American-British type, significantly different from the reference cohort. Erythrophagocytosis (70%), leukemia cutis (58%), and disseminated intravascular coagulation (39%) occurred frequently. Strikingly, spontaneous remissions occurred in 7 neonates with t(8;16)(p11;p13), of whom 3 remain in continuous remission. The 5-year overall survival of patients diagnosed after 1993 was 59%, similar to the reference cohort (P = .14). Gene expression profiles of t(8;16)(p11;p13) pediatric AML cases clustered close to, but distinct from, MLL-rearranged AML. Highly expressed genes included HOXA11, HOXA10, RET, PERP, and GGA2. In conclusion, pediatric t(8;16)(p11;p13) AML is a rare entity defined by a unique gene expression signature and distinct clinical features in whom spontaneous remissions occur in a subset of neonatal cases.
dcterms:title
Pediatric acute myeloid leukemia with t(8;16)(p11;p13): a distinct clinical and biological entity, a collaborative study by the International-Berlin-Frankfurt-Munster AML-study group. Pediatric acute myeloid leukemia with t(8;16)(p11;p13): a distinct clinical and biological entity, a collaborative study by the International-Berlin-Frankfurt-Munster AML-study group.
skos:prefLabel
Pediatric acute myeloid leukemia with t(8;16)(p11;p13): a distinct clinical and biological entity, a collaborative study by the International-Berlin-Frankfurt-Munster AML-study group. Pediatric acute myeloid leukemia with t(8;16)(p11;p13): a distinct clinical and biological entity, a collaborative study by the International-Berlin-Frankfurt-Munster AML-study group.
skos:notation
RIV/61989592:15110/13:33144176!RIV14-MSM-15110___
n5:predkladatel
n6:orjk%3A15110
n3:aktivita
n12:I
n3:aktivity
I
n3:cisloPeriodika
15
n3:dodaniDat
n18:2014
n3:domaciTvurceVysledku
n11:8117284
n3:druhVysledku
n7:J
n3:duvernostUdaju
n16:S
n3:entitaPredkladatele
n17:predkladatel
n3:idSjednocenehoVysledku
95653
n3:idVysledku
RIV/61989592:15110/13:33144176
n3:jazykVysledku
n4:eng
n3:klicovaSlova
HOXA11, HOXA10, RET, PERP, and GGA2; p13); 16)(p11; gene t(8; acute myeloid leukemia (AML)
n3:klicoveSlovo
n14:RET n14:p13%29 n14:acute%20myeloid%20leukemia%20%28AML%29 n14:gene%20t%288 n14:HOXA11 n14:and%20GGA2 n14:16%29%28p11 n14:HOXA10 n14:PERP
n3:kodStatuVydavatele
US - Spojené státy americké
n3:kontrolniKodProRIV
[D2D014D6C2B3]
n3:nazevZdroje
Blood (online)
n3:obor
n15:FD
n3:pocetDomacichTvurcuVysledku
1
n3:pocetTvurcuVysledku
26
n3:rokUplatneniVysledku
n18:2013
n3:svazekPeriodika
122
n3:tvurceVysledku
Mihál, Vladimír Coenen, Eva
s:issn
1528-0020
s:numberOfPages
10
n19:doi
10.1182/blood-2013-02-485524
n8:organizacniJednotka
15110