T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management

T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Ch...

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Autores principales: Parveen Shiraz, Waqas Jehangir, Vaibhav Agrawal
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Publicado: MDPI AG 2021
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spelling oai:doaj.org-article:cbf3dcaa43fb42edb04875642153b60d2021-11-25T16:49:40ZT-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management10.3390/biomedicines91116212227-9059https://doaj.org/article/cbf3dcaa43fb42edb04875642153b60d2021-11-01T00:00:00Zhttps://www.mdpi.com/2227-9059/9/11/1621https://doaj.org/toc/2227-9059T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intensive regimens and have superior outcomes compared to older adults. In children and young adults, Nelarabine added to frontline chemotherapy improves outcomes and end of consolidation measurable residual disease has emerged as the most valuable prognostic marker. While outcomes for de-novo disease are steadily improving, patients with relapsed and refractory T-ALL fare poorly. Newer targeted therapies are being studied in large clinical trials and have the potential to further improve outcomes. The role of allogeneic stem cell transplant (HSCT) is evolving due to the increased use of pediatric-inspired regimens and MRD monitoring. In this review we will discuss the biology, treatment, and outcomes in pediatric and adult T-ALL.Parveen ShirazWaqas JehangirVaibhav AgrawalMDPI AGarticleacute lymphoblastic leukemiaT-cell leukemiaNOTCHNelarabineBiology (General)QH301-705.5ENBiomedicines, Vol 9, Iss 1621, p 1621 (2021)
institution DOAJ
collection DOAJ
language EN
topic acute lymphoblastic leukemia
T-cell leukemia
NOTCH
Nelarabine
Biology (General)
QH301-705.5
spellingShingle acute lymphoblastic leukemia
T-cell leukemia
NOTCH
Nelarabine
Biology (General)
QH301-705.5
Parveen Shiraz
Waqas Jehangir
Vaibhav Agrawal
T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
description T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intensive regimens and have superior outcomes compared to older adults. In children and young adults, Nelarabine added to frontline chemotherapy improves outcomes and end of consolidation measurable residual disease has emerged as the most valuable prognostic marker. While outcomes for de-novo disease are steadily improving, patients with relapsed and refractory T-ALL fare poorly. Newer targeted therapies are being studied in large clinical trials and have the potential to further improve outcomes. The role of allogeneic stem cell transplant (HSCT) is evolving due to the increased use of pediatric-inspired regimens and MRD monitoring. In this review we will discuss the biology, treatment, and outcomes in pediatric and adult T-ALL.
format article
author Parveen Shiraz
Waqas Jehangir
Vaibhav Agrawal
author_facet Parveen Shiraz
Waqas Jehangir
Vaibhav Agrawal
author_sort Parveen Shiraz
title T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
title_short T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
title_full T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
title_fullStr T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
title_full_unstemmed T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
title_sort t-cell acute lymphoblastic leukemia—current concepts in molecular biology and management
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/cbf3dcaa43fb42edb04875642153b60d
work_keys_str_mv AT parveenshiraz tcellacutelymphoblasticleukemiacurrentconceptsinmolecularbiologyandmanagement
AT waqasjehangir tcellacutelymphoblasticleukemiacurrentconceptsinmolecularbiologyandmanagement
AT vaibhavagrawal tcellacutelymphoblasticleukemiacurrentconceptsinmolecularbiologyandmanagement
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