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SET-NUP214 is a recurrent γδ lineage-specific fusion transcript associated with corticosteroid/chemotherapy resistance in adult T-ALL
- Source :
- Blood. 123:1860-1863
- Publication Year :
- 2014
- Publisher :
- American Society of Hematology, 2014.
-
Abstract
- The SET-NUP214 (TAF1/CAN) fusion gene is a rare genetic event in T-cell acute lymphoblastic leukemia (T-ALL). Eleven (6%) of 196 T-ALL patients enrolled in the French Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL) 2003 and 2005 trials harbored a SET-NUP214 transcript. SET-NUP214-positive patients were predominantly (10 [91%] of 11) T-cell receptor (TCR)-negative and strikingly associated with TCRγδ lineage T-ALLs, as defined by expression of TCRγδ, TCRδ and/or TCRγ rearrangements but no complete TCRβ variable diversity joining rearrangement in surface CD3/TCR-negative cases. When compared with SET-NUP214-negative patients, SET-NUP214-positive patients showed a significantly higher rate of corticosteroid resistance (91% vs 44%; P = .003) and chemotherapy resistance (100% vs 44%; P = .0001). All SET-NUP214-positive patients but one achieved complete remission, and 9 were allografted. Despite the poor early-treatment sensitivity, the outcome of SET-NUP214-positive patients was similar to that of SET-NUP214-negative patients.
- Subjects :
- Adult
Male
T cell
CD3
Gene Rearrangement, delta-Chain T-Cell Antigen Receptor
Immunology
Antineoplastic Agents
Drug resistance
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Biochemistry
Fusion gene
Young Adult
Adrenal Cortex Hormones
Humans
Medicine
Histone Chaperones
Oncogene Fusion
biology
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
business.industry
T-cell receptor
Receptors, Antigen, T-Cell, gamma-delta
Cell Biology
Hematology
Gene rearrangement
Middle Aged
DNA-Binding Proteins
Nuclear Pore Complex Proteins
medicine.anatomical_structure
Fusion transcript
Drug Resistance, Neoplasm
Adult Acute Lymphoblastic Leukemia
biology.protein
Female
business
Transcription Factors
Subjects
Details
- ISSN :
- 15280020 and 00064971
- Volume :
- 123
- Database :
- OpenAIRE
- Journal :
- Blood
- Accession number :
- edsair.doi.dedup.....8d2762d5406fd29c4d10c1502f7addb8