The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.
Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia
Open accessAbstract
Background Approximately one-fifth of children with acute T-lymphoblastic leukemia (T-ALL) succumb to the disease, suggesting unrecognized biologic heterogeneity that may contribute to drug resistance. We hypothesized that T-ALL originating from early T-cell precursors (ETPs), a recently defined subset of thymocytes that retain stem cell-like features, would respond poorly to lymphoid-cell directed therapy. We studied leukemic cells, collected at diagnosis, to identify cases with ETP features and determine their clinical outcome. Methods Leukemic cells from 239 patients with T-ALL enrolled at St. Jude and in the Italian national study AIEOP ALL-2000 were examined by gene expression profiling, flow cytometry and single nucleotide polymorphism array analysis. Probabilities of survival and treatment failure were calculated for subgroups considered to have ETP-ALL or typical T-ALL. Findings Thirty patients (12.6%) had leukemic lymphoblasts with an ETP-related gene expression signature or its associated distinctive immunophenotype (CD1a−, CD8−, CD5weak with stem-cell/myeloid markers). Cases of ETP-ALL showed increased genomic instability. Patients with this form of leukemia had a very high proportion of remission failure or hematologic relapse: 72% (95% confidence interval, 40% to 100%) at 10 years versus 10% (4% to 16%) for typical TALL patients treated at St. Jude; and 57% (25% to 89%) at 2 years versus 14% (6% to 22%) for patients treated in the AIEOP trial. Interpretation ETP-ALL is a distinct, previously unrecognized, pathobiologic entity that confers a dire prognosis with use of standard intensive chemotherapy. Its early recognition, using the criteria outlined here, is essential for the development of an effective clinical management strategy.
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Self-citationGenome-wide analysis of genetic alterations in acute lymphoblastic leukaemia
Mullighan, C. G., Goorha, S., Radtke, I., Miller, C. B., Coustan-Smith, E., Dalton, J. D., Girtman, K., Mathew, S., Ma, J., Pounds, S. B., et al. (2007) · Nature · doi:10.1038/nature05690 · Cited by 1,801
Self-citationGene expression signatures define novel oncogenic pathways in T cell acute lymphoblastic leukemia
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The genetic basis of early T-cell precursor acute lymphoblastic leukaemia
Zhang, J., Ding, L., Holmfeldt, L., Wu, G., Heatley, S. L., Payne-Turner, D., Easton, J., Chen, X., Wang, J., Rusch, M., et al. (2012) · Nature · doi:10.1038/nature10725 · Cited by 1,596
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Self-citationThe genomic landscape of pediatric and young adult T-lineage acute lymphoblastic leukemia
Liu, Y., Easton, J., Shao, Y., Maciaszek, J., Wang, Z., Wilkinson, M. R., McCastlain, K., Edmonson, M., Pounds, S. B., Shi, L., et al. (2017) · Nature Genetics · doi:10.1038/ng.3909 · Cited by 918
Self-citationHomminga, I., Pieters, R., Langerak, A. W., de Rooi, J. J., Stubbs, A., Verstegen, M., Vuerhard, M., Buijs-Gladdines, J., Kooi, C., Klous, P., et al. (2011) · Cancer Cell · doi:10.1016/j.ccr.2011.02.008 · Cited by 384
Metadata
- Authors
- Coustan-Smith, E., Mullighan, C. G., Onciu, M., Behm, F. G., Raimondi, S. C., Pei, D., Cheng, C., Su, X., Rubnitz, J. E., Basso, G., Biondi, A., Pui, C.-H., Downing, J. R., & Campana, D.
- Venue
- The Lancet Oncology
- Publisher
- Elsevier BV
- Published
- 2009-02
- Volume
- 10
- Issue
- 2
- Pages
- 147-156
- ISSN
- 1470-2045 (ISSN-L)
- OpenAlex
- W2166306614
- Cited by
- 1,019
- References
- 33
- FWCI
- 41.99
- Type
- journal article
- Language
- en
- Open access
- green
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- Coustan-Smith, E., Mullighan, C. G., Onciu, M., Behm, F. G., Raimondi, S. C., Pei, D., Cheng, C., Su, X., Rubnitz, J. E., Basso, G., Biondi, A., Pui, C.-H., Downing, J. R., & Campana, D. (2009). Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia. The Lancet Oncology. https://doi.org/10.1016/s1470-2045(08)70314-0
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