Recurrent abnormalities can be used for risk group stratification in pediatric AMKL: a retrospective intergroup study

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F16%3A10324733" target="_blank" >RIV/00064203:_____/16:10324733 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11130/16:10324733

Result on the web

<a href="http://dx.doi.org/10.1182/blood-2016-01-695551" target="_blank" >http://dx.doi.org/10.1182/blood-2016-01-695551</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1182/blood-2016-01-695551" target="_blank" >10.1182/blood-2016-01-695551</a>

Result language

angličtina

Original language name

Recurrent abnormalities can be used for risk group stratification in pediatric AMKL: a retrospective intergroup study

Original language description

Genetic abnormalities and early treatment response are the main prognostic factors in acute myeloid leukemia (AML). Acute megakaryoblastic leukemia (AMKL) is a rare subtype of AML. Deep sequencing has identified CBFA2T3/GLIS2 and NUP98/KDM5A as recurrent aberrations, occurring in similar frequencies as RBM15/MKL1 and KMT2A-rearrangements. We studied whether these cytogenetic aberrations can be used for risk group stratification. To assess frequencies and outcome parameters of recurrent cytogenetic aberrations in AMKL, samples and clinical data of patients treated by the Associazione Italiana Ematologia Oncologia Pediatrica, Berlin-Frankfurt-Munster Study Group, Children's Oncology Group, Dutch Childhood Oncology Group, and the Saint Louis Hopital were collected, enabling us to screen 153 newly diagnosed pediatric AMKL cases for the aforementioned aberrations and to study their clinical characteristics and outcome. CBFA2T3/GLIS2 was identified in 16% of the cases; RBM15/MKL1, in 12%; NUP98/KDM5A and KMT2A rearrangements, in 9% each; and monosomy 7, in 6%. These aberrations were mutually exclusive. RBM15/MKL1-rearranged patients were significantly younger. No significant differences in sex and white blood cell count were found. NUP98/KDM5A, CBFA2T3/GLIS2, KMT2A-rearranged lesions and monosomy 7 (NCK-7) independently predicted a poor outcome, compared with RBM15/MKL1-rearranged patients and those with AMKL not carrying these molecular lesions. NCK-7-patients (n=61) showed a 4-year probability of overall survival of 35 6 6% vs 70 +/- 5% in the RBM15/MKL1-other groups (n = 92, P<.0001) and 4-year probability of event-free survival of 33 +/- 6% vs 62 +/- 5% (P=.0013), the 4-year cumulative incidence of relapse being 42 +/- 7% and 19 +/- 4% (P=.003), respectively. We conclude that these genetic aberrations may be used for risk group stratification of pediatric AMKL and for treatment tailoring.

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FD - Oncology and haematology

OECD FORD branch

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Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2016

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Blood

ISSN

0006-4971

e-ISSN

—

Volume of the periodical

127

Issue of the periodical within the volume

26

Country of publishing house

US - UNITED STATES

Number of pages

7

Pages from-to

3424-3430

UT code for WoS article

000379248500018

EID of the result in the Scopus database

2-s2.0-84977278887