2015
DOI: 10.1182/blood.v126.23.1418.1418
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Early T-Cell Precursor Acute Lymphoblastic Leukemia (ETP-ALL) Is a High-Risk Subtype in Adults

Abstract: Background: Early T-cell precursor (ETP) acute lymphoblastic leukemia/lymphoma (ALL) is a recently recognized high-risk T-ALL subgroup. The optimal therapeutic approaches to adult patients with ETP-ALL are poorly characterized. In this study, we compared the outcomes of adults with ETP-ALL who received treatment on frontline regimens to those of patients with other T-ALL immunophenotypic subtypes. Methods: Patients with newly-diagnosed T-ALL who received frontline chemotherapy between the years … Show more

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“…Genetically, ETP-ALL frequently associates with mutations in genes encoding epigenetic regulators ( IDH1, IDH2, and DNMT3A ), signaling factors (i.e., NRAS and FLT3 ), and transcription factors involved in hematopoietic and T-cell development ( RUNX1, GATA3 and ETV6 ), whereas both activating NOTCH1 mutations and CDKN2A deletions co-occurring with oncogenic NOTCH1 mutations are rarely observed [ 26 , 27 ]. ETP-ALL has been for years associated with poor prognosis [ 25 , 28 , 29 ], but application of early response-based intensification regimens in the last years has greatly improved the outcome of these patients [ 30 , 31 ].…”
Section: Introductionmentioning
confidence: 99%
“…Genetically, ETP-ALL frequently associates with mutations in genes encoding epigenetic regulators ( IDH1, IDH2, and DNMT3A ), signaling factors (i.e., NRAS and FLT3 ), and transcription factors involved in hematopoietic and T-cell development ( RUNX1, GATA3 and ETV6 ), whereas both activating NOTCH1 mutations and CDKN2A deletions co-occurring with oncogenic NOTCH1 mutations are rarely observed [ 26 , 27 ]. ETP-ALL has been for years associated with poor prognosis [ 25 , 28 , 29 ], but application of early response-based intensification regimens in the last years has greatly improved the outcome of these patients [ 30 , 31 ].…”
Section: Introductionmentioning
confidence: 99%