| Autor: |
Sendker, Stephanie, Awada, Amani, Domagalla, Sophia, Sendker, Michael, Orhan, Eser, Hoffmeister, Lina Marie, Antoniou, Evangelia, Niktoreh, Naghmeh, Reinhardt, Dirk, von Neuhoff, Nils, Schneider, Markus |
| Zdroj: |
Leukemia; July 2023, Vol. 37 Issue: 7 p1435-1443, 9p |
| Abstrakt: |
In acute myeloid leukaemia (AML) RUNX1mutation is characterised by certain clinicopathological features with poor prognosis and adverse risk by the European LeukemiaNet recommendation. Though initially considered as provisional category, the recent World Health Organisation (WHO) classification of 2022 removed RUNX1-mutated AML from the unique entity. However, the significance of RUNX1mutation in paediatric AML remains unclear. We retrospectively analysed a German cohort of 488 paediatric patients with de novo AML, enroled in the AMLR12 or AMLR17 registry of the AML-BFM Study Group (Essen, Germany). A total of 23 paediatric AML patients (4.7%) harboured RUNX1mutations, 18 of which (78%) had RUNX1mutation at initial diagnosis. RUNX1mutations were associated with older age, male gender, number of coexisting alterations and presence of FLT3-ITD but mutually exclusive of KRAS, KITand NPM1mutation. RUNX1mutations did not prognostically impact overall or event-free survival. Response rates did not differ between patients with and without RUNX1mutations. This comprehensive study, comprising the largest analysis of RUNX1mutation in a paediatric cohort to date, reveals distinct but not unique clinicopathologic features, with no prognostic significance of RUNX1-mutated paediatric AML. These results broaden the perspective on the relevance of RUNX1alterations in leukaemogenesis in AML. |
| Databáze: |
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