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UBTFtandem duplications define a distinct subtype of adult de novo acute myeloid leukemia
- Source :
- Leukemia; June 2023, Vol. 37 Issue: 6 p1245-1253, 9p
- Publication Year :
- 2023
-
Abstract
- Tandem duplications (TDs) of the UBTFgene have been recently described as a recurrent alteration in pediatric acute myeloid leukemia (AML). Here, by screening 1946 newly diagnosed adult AML, we found that UBTF-TDs occur in about 3% of patients aged 18–60 years, in a mutually exclusive pattern with other known AML subtype-defining alterations. The characteristics of 59 adults with UBTF-TD AML included young age (median 37 years), low bone marrow (BM) blast infiltration (median 25%), and high rates of WT1mutations (61%), FLT3-ITDs (51%) and trisomy 8 (29%). BM morphology frequently demonstrates dysmyelopoiesis albeit modulated by the co-occurrence of FLT3-ITD. UBTF-TD patients have lower complete remission (CR) rates (57% after 1 course and 76% after 2 courses of intensive chemotherapy [ICT]) than UBTF-wild-type patients. In patients enrolled in the ALFA-0702 study (n= 614 patients including 21 with UBTF-TD AML), the 3-year disease-free survival (DFS) and overall survival of UBTF-TD patients were 42.9% (95%CI: 23.4–78.5%) and 57.1% (95%CI: 39.5–82.8%) and did not significantly differ from those of ELN 2022 intermediate/adverse risk patients. Finally, the study of paired diagnosis and relapsed/refractory AML samples suggests that WT1-mutated clones are frequently selected under ICT. This study supports the recognition of UBTF-TD AML as a new AML entity in adults.
Details
- Language :
- English
- ISSN :
- 08876924 and 14765551
- Volume :
- 37
- Issue :
- 6
- Database :
- Supplemental Index
- Journal :
- Leukemia
- Publication Type :
- Periodical
- Accession number :
- ejs62886149
- Full Text :
- https://doi.org/10.1038/s41375-023-01906-z