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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2021
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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) |
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acute lymphoblastic leukemia T-cell leukemia NOTCH Nelarabine Biology (General) QH301-705.5 |
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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 |
_version_ |
1718412923765784576 |