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Lookup NU author(s): Professor Christine Harrison FRCPath FMedSci
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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.
Author(s): Coenen EA, Zwaan CM, Reinhardt D, Harrison CJ, Haas OA, de Haas V, Mihal V, De Moerloose B, Jeison M, Rubnitz JE, Tomizawa D, Johnston D, Alonzo TA, Hasle H, Auvrignon A, Dworzak M, Pession A, van der Velden VHJ, Swansbury J, Wong KF, Terui K, Savasan S, Winstanley M, Vaitkeviciene G, Zimmermann M, Pieters R, van den Heuvel-Eibrink MM
Publication type: Article
Publication status: Published
Print publication date: 10/10/2013
Online publication date: 23/08/2013
Acceptance date: 22/07/2013
ISSN (print): 0006-4971
ISSN (electronic): 1528-0020
Publisher: American Society of Hematology
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