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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. CDKN2A/CDKN2B and NOTCH1 are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intens...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615775/ https://www.ncbi.nlm.nih.gov/pubmed/34829849 http://dx.doi.org/10.3390/biomedicines9111621 |
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author | Shiraz, Parveen Jehangir, Waqas Agrawal, Vaibhav |
author_facet | Shiraz, Parveen Jehangir, Waqas Agrawal, Vaibhav |
author_sort | Shiraz, Parveen |
collection | PubMed |
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. CDKN2A/CDKN2B and NOTCH1 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 | Online Article Text |
id | pubmed-8615775 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-86157752021-11-26 T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management Shiraz, Parveen Jehangir, Waqas Agrawal, Vaibhav Biomedicines Review 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. CDKN2A/CDKN2B and NOTCH1 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. MDPI 2021-11-04 /pmc/articles/PMC8615775/ /pubmed/34829849 http://dx.doi.org/10.3390/biomedicines9111621 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Shiraz, Parveen Jehangir, Waqas Agrawal, Vaibhav T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
title | 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_short | 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 |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615775/ https://www.ncbi.nlm.nih.gov/pubmed/34829849 http://dx.doi.org/10.3390/biomedicines9111621 |
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