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.
abstract: 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.
authors: Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands;
authors: Eva A
authors: EA
authors: Coenen
authors: C Michel
authors: CM
authors: Zwaan
authors: Dirk
authors: D
authors: Reinhardt
authors: Christine J
authors: CJ
authors: Harrison
authors: Oskar A
authors: OA
authors: Haas
authors: Valerie
authors: V
authors: de Haas
authors: Vladimir
authors: V
authors: Mihál
authors: Barbara
authors: B
authors: De Moerloose
authors: Marta
authors: M
authors: Jeison
authors: Jeffrey E
authors: JE
authors: Rubnitz
authors: Daisuke
authors: D
authors: Tomizawa
authors: Donna
authors: D
authors: Johnston
authors: Todd A
authors: TA
authors: Alonzo
authors: Henrik
authors: H
authors: Hasle
authors: Anne
authors: A
authors: Auvrignon
authors: Michael
authors: M
authors: Dworzak
authors: Andrea
authors: A
authors: Pession
authors: Vincent H J
authors: VH
authors: van der Velden
authors: John
authors: J
authors: Swansbury
authors: Kit-fai
authors: KF
authors: Wong
authors: Kiminori
authors: K
authors: Terui
authors: Sureyya
authors: S
authors: Savasan
authors: Mark
authors: M
authors: Winstanley
authors: Goda
authors: G
authors: Vaitkeviciene
authors: Martin
authors: M
authors: Zimmermann
authors: Rob
authors: R
authors: Pieters
authors: Marry M
authors: MM
authors: van den Heuvel-Eibrink
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002885:Chromosomes, Human, Pair 16; D002898:Chromosomes, Human, Pair 8; D003299:Cooperative Behavior; D005260:Female; D015973:Gene Expression Regulation, Leukemic; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015470:Leukemia, Myeloid, Acute / Q000235:genetics* / Q000401:mortality* / Q000628:therapy; D008297:Male; D011379:Prognosis; D012075:Remission, Spontaneous; D059467:Transcriptome; D014178:Translocation, Genetic / Q000235:genetics*
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