In:
Blood Cancer Journal, Springer Science and Business Media LLC, Vol. 8, No. 9 ( 2018-08-31)
Abstract:
Mutations of the GATA binding protein 2 ( GATA2 ) gene in myeloid malignancies usually cluster in the zinc finger 1 (ZF1) and the ZF2 domains. Mutations in different locations of GATA2 may have distinct impact on clinico-biological features and outcomes in AML patients, but little is known in this aspect. In this study, we explored GATA2 mutations in 693 de novo non-M3 AML patients and identified 44 GATA2 mutations in 43 (6.2%) patients, including 31 in ZF1, 10 in ZF2, and three outside the two domains. Different from GATA2 ZF2 mutations, ZF1 mutations were closely associated with French-American-British (FAB) M1 subtype, CEBPA double mutations ( CEBPA double-mut ), but inversely correlated with FAB M4 subtype, NPM1 mutations, and FLT3 -ITD. ZF1-mutated AML patients had a significantly longer overall survival (OS) than GATA2- wild patients and ZF2-mutated patients in total cohort as well as in those with intermediate-risk cytogenetics and normal karyotype. ZF1 mutations also predicted better disease-free survival and a trend of better OS in CEBPA double-mut patients. Sequential analysis showed GATA2 mutations could be acquired at relapse. In conclusion, GATA2 ZF1 mutations are associated with distinct clinico-biological features and predict better prognosis, different from ZF2 mutations, in AML patients.
Type of Medium:
Online Resource
ISSN:
2044-5385
DOI:
10.1038/s41408-018-0123-2
Language:
English
Publisher:
Springer Science and Business Media LLC
Publication Date:
2018
detail.hit.zdb_id:
2600560-8
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