Pediatric acute myeloid leukemia with t(8;16)(p11;p13), a distinct clinical and biological entity: A collaborative study by the International-Berlin- Frankfurt-Münster AML-study group

Eva A. Coenen, C. Michel Zwaan, Dirk Reinhardt, Christine J. Harrison, Oskar A. Haas, Valerie De Haas, Vladimir Mihál, Barbara De Moerloose, Marta Jeison, Jeffrey E. Rubnitz, Daisuke Tomizawa, Donna Johnston, Todd A. Alonzo, Henrik Hasle, Anne Auvrignon, Michael Dworzak, Andrea Pession, Vincent H.J. Van Der Velden, John Swansbury, Kit Fai WongKiminori Terui, Sureyya Savasan, Mark Winstanley, Goda Vaitkeviciene, Martin Zimmermann, Rob Pieters, Marry M. Van Den Heuvel-Eibrink

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63 Scopus citations

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.

Original languageEnglish
Pages (from-to)2704-2713
Number of pages10
JournalBlood
Volume122
Issue number15
DOIs
StatePublished - Oct 10 2013

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