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Acute myeloid leukemia (AML) with t(7;12)(q36;p13) is associated with infancy and trisomy 19: Data from Nordic Society for Pediatric Hematology and Oncology (NOPHO-AML) and review of the literature

Artikel i vetenskaplig tidskrift
Författare A. D. L. Espersen
U. Noren-Nystrom
Jonas Abrahamsson
S. Y. Ha
C. J. Pronk
K. Jahnukainen
O. G. Jonsson
B. Lausen
J. Palle
B. Zeller
Lars Palmqvist
H. Hasle
Publicerad i Genes Chromosomes & Cancer
Volym 57
Nummer/häfte 7
Sidor 359-365
ISSN 1045-2257
Publiceringsår 2018
Publicerad vid Institutionen för biomedicin, avdelningen för klinisk kemi och transfusionsmedicin
Sidor 359-365
Språk en
Länkar doi.org/10.1002/gcc.22538
Ämnesord acute leukemia, AML, cytogenetics, pediatric, t(7, 12)(q36, p13), ectopic expression, clinical-features, children, therapy, hlxb9, etv6, translocations, heterogeneity, diagnosis, trials
Ämneskategorier Hematologi, Cancer och onkologi

Sammanfattning

The t(7;12)(q36;p13) (MNX1/ETV6) is not included in the WHO classification but has been described in up to 30% of acute myeloid leukemia (AML) in children <2 years and associated with a poor prognosis. We present the clinical and cytogenetics characteristics of AML cases with t(7;12)(p36;p13). A literature review identified 35 patients with this translocation, published between 2000 and 2015. Outcome data were available in 22 cases. The NOPHO-AML (Nordic Society for Pediatric Hematology and Oncology) database contained 651 patients with AML from 1993 to 2014 and seven (1.1%) had the translocation. The t(7;12) was only present in patients <2 years of age (median age 6 months) but none was diagnosed as newborn. These patients constituted 4.3% of the patients <2 years of age. There was a strong association with trisomy 19 (literature: 86%, NOPHO: 100%) and +8 (literature: 19%, NOPHO: 14%). Seventeen of 22 patients from the literature with t(7;12) and four of seven patients from the NOPHO database suffered from relapse. The patients with t(7;12) had a 3-year event free survival of 24% (literature) vs. 43% (NOPHO) and a 3-year overall survival of 42% (literature) vs. 100% (NOPHO). None of the NOPHO patients was treated with hematopoietic stem cell transplantation (HSCT) in first complete remission. Relapse was frequent but the salvage rate using HSCT was high. We conclude that t(7;12)(q36;13) is a unique subgroup of childhood AML with presentation before 2 years of age with most cases being associated with +19.

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