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

Eva Coenen, C.M. Zwaan, D Reinhardt, CJ Harrison, OA Haas, V de Haas, V Mihal, B de Moerloose, M Jeison, JE Rubnitz, D Tomizawa, D Johnston, TA Alonzo, H Hasle, A Auvrignon, M Dworzak, A Pession, Vincent van der Velden, J Swansbury, KF WongK Terui, S Savasan, M Winstanley, G Vaitkeviciene, M Zimmermann, Rob Pieters, Marry Van den Heuvel - Eibrink

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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-Munster (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 languageUndefined/Unknown
Pages (from-to)2704-2713
Number of pages10
JournalBlood
Volume122
Issue number15
DOIs
Publication statusPublished - 2013

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