Clinical characteristics and outcomes of B-ALL with ZNF384 rearrangements: a retrospective analysis by the Ponte di Legno Childhood ALL Working Group

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F21%3A10425584" target="_blank" >RIV/00216208:11130/21:10425584 - isvavai.cz</a>

Alternative codes found

RIV/00064203:_____/21:10425584

Result on the web

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=t2RcW1OjVZ" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=t2RcW1OjVZ</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1038/s41375-021-01199-0" target="_blank" >10.1038/s41375-021-01199-0</a>

Result language

angličtina

Original language name

Clinical characteristics and outcomes of B-ALL with ZNF384 rearrangements: a retrospective analysis by the Ponte di Legno Childhood ALL Working Group

Original language description

ZNF384 fusions are an enigmatic group of gene fusions which span the ALL-MPAL disease spectrum and are not readily detected and characterized by traditional genetic testing.It is definitey required to screen by FISH, reverse transcription polymerase chain reaction or RNA sequencing. The major strengthen of this study is that it has collated a large well-annoatated cohort of ZNF384-fusion patients which while not uninformly treated were all diagnosed with ALL and treated as such. As with all retrospective consortia-based studies the limitations are heterogeneity in terms of recrtuiment period and treatment decisions and pathways. Nonetheless, we provide good evidence that among B-ALL patients with a ZNF384 fusion the partner gene is associated with demographic features and influences outcome, specifically with EP300-ZNF384 being associated with a lower risk of relapse. We opted to exlude MPAL cases because, historically, they have not been treated uniformly. A recent international cooperative study has defined a consensus treatment staregy for MPAL patients. This initiative coupled with improved diagnostic genomic testing will enable future prospective studies to clarify the clinical relevance of the fusions in both ALL and MPAL.

Czech name

—

Czech description

—

Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30205 - Hematology

Project

<a href="/en/project/NU20-07-00322" target="_blank" >NU20-07-00322: Genomics for the modern diagnostics and treatment of childhood leukaemias</a><br>

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year

2021

Confidentiality

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

Name of the periodical

Leukemia

ISSN

0887-6924

e-ISSN

—

Volume of the periodical

35

Issue of the periodical within the volume

11

Country of publishing house

GB - UNITED KINGDOM

Number of pages

6

Pages from-to

3272-3277

UT code for WoS article

000627262700003

EID of the result in the Scopus database

2-s2.0-85102241429