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Pediatric T-Cell Acute Lymphobastic Leukemia

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Pediatric Acute Lymphoblastic Leukemia

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Abstract

T-cell acute lymphoblastic leukemia (T-ALL), which accounts for 7–15% of pediatric ALL, has a distinct biology from B-cell precursor ALL (BCP-ALL). Despite improvements achieved with treatment-intensification strategies, compared with patients with BCP-ALL, the outcomes of patients with T-ALL are inferior. Studies have identified the genetic alterations underpinning T-ALL, defining subgroups with oncogenic transcription factor dysregulation and mutations or deletions leading to aberrant signaling pathway activation. Early T-cell precursor ALL is a recently defined subtype with unique immunophenotypic and genetic features. However, regarding prognostic significance, minimal residual disease (MRD), rather than genetic subgroups, is the most reliable indicator of T-ALL. Recent clinical trials have been designed to incorporate several key interventions—such as those with respect to dexamethasone use in induction, intensive l-asparaginase, high-dose methotrexate, and nelarabine—into MRD-directed treatments. Several studies omit cranial radiotherapy even for patients with central nervous system involvement at diagnosis, thus avoiding long-term adverse events. Progress in knowledge of tumor biology will lead to the development and use of new target therapies directed at genetic alterations, such as ABL1 fusions and aberrant activation of Notch1 or JAK-STAT pathways, via new approaches potentially improving the outcomes of pediatric T-ALL patients.

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Authors and Affiliations

  1. Department of Hematology and Oncology, Miyagi Children’s Hospital, Sendai, Japan

    Atsushi Sato

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  1. Atsushi Sato
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Correspondence to Atsushi Sato .

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  1. Department of Transplantation and Cell Therapy, Children’s Cancer, Center National Center for Child Health and Development, Setagaya-ku, Tokyo, Japan

    Motohiro Kato

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Sato, A. (2020). Pediatric T-Cell Acute Lymphobastic Leukemia. In: Kato, M. (eds) Pediatric Acute Lymphoblastic Leukemia. Springer, Singapore. https://doi.org/10.1007/978-981-15-0548-5_7

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  • DOI: https://doi.org/10.1007/978-981-15-0548-5_7

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