Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a unique subtype of immature T-cell ALL that was initially associated with a dramatically inferior prognosis compared with non-ETP T-cell ALL (Not-ETP) when it was first described in 2009. Analyses of larger patient cohorts treated with more contemporary regimens, however, have shown minimal survival differences between ETP and Not-ETP. In this manuscript, we use representative cases to explore therapeutic advances and address common clinical questions regarding the management of children, adolescents, and young adults with ETP-ALL. We describe our recommended treatment approach for a child or adolescent with newly diagnosed ETP-ALL, with an emphasis on the prognostic significance of induction failure and detectable minimal residual disease and the role of hematopoietic stem cell transplant in first remission. We discuss the interplay between the ETP immunophenotype and genomic markers of immaturity in T-cell ALL. Finally, we review novel therapeutic approaches that should be considered when managing relapsed or refractory ETP-ALL.
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Acute Lymphoblastic Leukemia|
January 2, 2025
How I treat ETP-ALL in children
Ryan J. Summers,
Ryan J. Summers
1Department of Pediatrics, Emory University, Atlanta, GA
2Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Atlanta, GA
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David T. Teachey,
David T. Teachey
3Department of Pediatrics and the Center for Childhood Cancer Research, Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA
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Stephen P. Hunger
Stephen P. Hunger
3Department of Pediatrics and the Center for Childhood Cancer Research, Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA
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Blood (2025) 145 (1): 43–52.
Article history
Submitted:
December 18, 2023
Accepted:
February 1, 2024
First Edition:
February 16, 2024
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Citation
Ryan J. Summers, David T. Teachey, Stephen P. Hunger; How I treat ETP-ALL in children. Blood 2025; 145 (1): 43–52. doi: https://doi.org/10.1182/blood.2023023155
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January 2 2025
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