Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies

Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a distinct subtype of T lymphoblastic leukemia (T-ALL) identified in 2009, due to its unique immunophenotypic and genomic profile. The outcome of patients was poor in earlier studies, and they were prone to have induction failure, with...

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Autores principales: Chun-fung Sin, Pui-hei Marcus Man
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Lenguaje:EN
Publicado: Frontiers Media S.A. 2021
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Acceso en línea:https://doaj.org/article/e6709299e1584832bc967422cf3e0cc3
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spelling oai:doaj.org-article:e6709299e1584832bc967422cf3e0cc32021-12-01T12:27:08ZEarly T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies2234-943X10.3389/fonc.2021.750789https://doaj.org/article/e6709299e1584832bc967422cf3e0cc32021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fonc.2021.750789/fullhttps://doaj.org/toc/2234-943XEarly T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a distinct subtype of T lymphoblastic leukemia (T-ALL) identified in 2009, due to its unique immunophenotypic and genomic profile. The outcome of patients was poor in earlier studies, and they were prone to have induction failure, with more frequent relapse/refractory disease. Recent advances had been made in discoveries of genetic aberrations and molecular pathogenesis of ETP-ALL. However, the diagnosis and management of ETP-ALL is still challenging. There are limited choices of novel therapies so far. In this review article, it highlighted the diagnostic issue of ETP-ALL, pitfall in diagnosis, and strategy of accurate diagnosis. The review also summarized current understanding of molecular mechanism of leukemogenesis. The emerging role of risk-adapted therapy and allogenic stem cell transplant in optimizing the outcome of patients with ETP-ALL was discussed. Finally, some potential novel therapies were proposed based on the current understanding of molecular pathogenesis.Chun-fung SinPui-hei Marcus ManFrontiers Media S.A.articleETP-ALLT lymphoblastic leukemiaearly T-cell precursor acute lymphoblastic leukemiadiagnosismolecular pathogenesisnovel therapiesNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENFrontiers in Oncology, Vol 11 (2021)
institution DOAJ
collection DOAJ
language EN
topic ETP-ALL
T lymphoblastic leukemia
early T-cell precursor acute lymphoblastic leukemia
diagnosis
molecular pathogenesis
novel therapies
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
spellingShingle ETP-ALL
T lymphoblastic leukemia
early T-cell precursor acute lymphoblastic leukemia
diagnosis
molecular pathogenesis
novel therapies
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
Chun-fung Sin
Pui-hei Marcus Man
Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
description Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a distinct subtype of T lymphoblastic leukemia (T-ALL) identified in 2009, due to its unique immunophenotypic and genomic profile. The outcome of patients was poor in earlier studies, and they were prone to have induction failure, with more frequent relapse/refractory disease. Recent advances had been made in discoveries of genetic aberrations and molecular pathogenesis of ETP-ALL. However, the diagnosis and management of ETP-ALL is still challenging. There are limited choices of novel therapies so far. In this review article, it highlighted the diagnostic issue of ETP-ALL, pitfall in diagnosis, and strategy of accurate diagnosis. The review also summarized current understanding of molecular mechanism of leukemogenesis. The emerging role of risk-adapted therapy and allogenic stem cell transplant in optimizing the outcome of patients with ETP-ALL was discussed. Finally, some potential novel therapies were proposed based on the current understanding of molecular pathogenesis.
format article
author Chun-fung Sin
Pui-hei Marcus Man
author_facet Chun-fung Sin
Pui-hei Marcus Man
author_sort Chun-fung Sin
title Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
title_short Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
title_full Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
title_fullStr Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
title_full_unstemmed Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies
title_sort early t-cell precursor acute lymphoblastic leukemia: diagnosis, updates in molecular pathogenesis, management, and novel therapies
publisher Frontiers Media S.A.
publishDate 2021
url https://doaj.org/article/e6709299e1584832bc967422cf3e0cc3
work_keys_str_mv AT chunfungsin earlytcellprecursoracutelymphoblasticleukemiadiagnosisupdatesinmolecularpathogenesismanagementandnoveltherapies
AT puiheimarcusman earlytcellprecursoracutelymphoblasticleukemiadiagnosisupdatesinmolecularpathogenesismanagementandnoveltherapies
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